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Soft Tissue Aneurysmal Bone Cyst: A Case Report

Posted on June 1, 2011 | Comments Off on Soft Tissue Aneurysmal Bone Cyst: A Case Report

by Kevin M McCann, DPM 1  , Craig E Clifford, DPM 2 , Heather L Salton, DPM, AACFAS 3 

The Foot and Ankle Online Journal 4 (6): 1

The authors present an unusual case of a primary soft tissue tumor having histologic features identical to an intraosseous aneurismal bone cyst. A retrospective chart and radiographic review of a 44 year-old male was performed with a 17 month follow-up. Initially presenting as a painful, palpable nodule on the medial left ankle; a discreet, encapsulated mass was confirmed with ultrasound and magnetic resonance imaging. Surgical excision followed and pathologic analysis of the specimen diagnosed an aneurysmal bone cyst in soft tissue. Aneurysmal bone cysts usually appear in the metaphyses of long bones or in vertebral bodies. The primary soft tissue tumor is rare, with only 17 cases having been reported in the English literature. This is the first report of a primary soft tissue aneurysmal bone cyst in the distal lower extremity. In this case, as in the majority of reported cases, complete resolution was obtained with surgical excision.

Key words: Aneurysmal Bone Cyst, Tumor, Magnetic Resonance Imaging, Case Report

Accepted: April, 2011
Published: June, 2011

ISSN 1941-6806
doi: 10.3827/faoj.2011.0406.0001

Aneurysmal bone cyst (ABC) is classically described as a benign lesion developing mostly in the metaphyses of long bones and in vertebral bodies. [1] They are characterized by blood-filled spaces separated by connective tissue septa containing fibroblasts, osteoclast-like giant cells, and reactive woven bone. [2] ABC is considered a non-neoplastic lesion, although some cytogenetic studies have found reproducible chromosomal abnormalities. [3,18] The majority of ABCs arise de novo (primary ABC), but some have been found associated with other bony lesions secondarily. [4,15]

Lesions usually appear on radiograph as lytic and expansile. Computed tomography (CT) or magnetic resonance imaging (MRI) modalities often show a heterogeneous lesion with fluid-filled cystic spaces surrounded by a thin rim of bone. [5] Standard treatment of ABCs with marginal, yet complete surgical excision has shown acceptable results. [3,5,6]

While traditionally classified as a more common benign tumor of bone, ABCs have rarely been described as being found in soft tissues. A search of the English literature found a total of 17 cases of soft tissue ABC, with no primary lesions previously described in the distal lower extremity. [3,4, 7-16]

Case Report

The patient is a 44 year-old male who originally presented with complaints of a palpable “knot” in his medial left ankle, separate from and incidental to his chief complaint of hallux limitus on the contralateral limb. Initial exam showed a firm, semi-mobile, deep mass approximately 2cm proximal and immediately posterior to the medial malleolus. This was mildly tender to palpation, but did not restrict motion of the lower extremity. Ultrasound exam revealed a 5 x 10mm mass posterior to the tibialis posterior tendon. The mass was heterogeneous and ill-defined, without evidence of attachment to adjacent structures.

After one month of observation, the patient expressed continued pain and possible increasing size. An MRI exam was performed to show a 1.8 x 1cm heterogeneous mass abutting the flexor digitorum longus tendon. (Figs. 1A, 1B and 1C) A decision was made to perform a complete excisional biopsy.

  

Figure 1A, 1B and 1C  Magnetic resonance images (MRI) showing the heterogeneous, well-encapsulated mass immediately posterior to the flexor digitorum longus tendon.  (A) Coronal Proton-dense T2.  (B)  Sagittal T1 image.   (C)  Axial T1 Image.

Surgical dissection revealed a firm, purplish-brown, well-encapsulated mass within membranous soft tissue and with no defined attachment to surrounding structures. (Fig. 2A)

The mass was tagged with suture to identify orientation and submitted to pathology as a single specimen. (Fig. 2B) The patient healed uneventfully with full weight-bearing in the immediate post-operative phase.

 

Figure 2A and 2B  Intra-operative (A) and gross specimen photos (B) showing the excised mass with attached membranous soft tissue, and its location posterior to the tibialis posterior tendon.

The specimen margins were inked per orientation and the mass was sectioned to reveal a “variegated red-yellow cut surface with calcified tissue and extensive hemorrhage.” Microscopic analysis proved inconclusive and slides were sent to an outside pathology lab for independent review by multiple pathologists. The final pathology report is as follows: “The specimen is a circumscribed nodular heterogeneous lesion.”

Hemosiderotic synovium and a pool of muscular-walled, variably sized vessels are seen at the periphery of the nodule. The nodule had a vaguely multilobulated configuration with blood-filled pseudocystic spaces alternating with spindle cell areas and areas of bone formation. The center of the lesion is occupied by well-formed bone with spicules that radiate outward. The outer rim of the nodule demonstrates a discontinuous thin shell of bone. Numerous giant cells are seen within the blood filled spaces. Prominent hemosiderin deposition is seen within the intervening spindle-cell areas. The histologic features are those of so-called aneurismal bone cyst of soft tissue. (Fig. 3) No neoplasm is seen. The patient remains without symptoms or signs of recurrence at a follow-up of 17 months post-operatively.

Figure 3   Prepared H&E stained slide showing histologic features of aneurysmal bone cyst in soft tissue at 40X magnification.

Discussion

Unlike the more common primary bone lesion, soft tissue aneurysmal bone cyst is a rarely occurring pathology. A search of English language databases revealed a total of 17 cases of soft tissue ABC as reported by 12 authors. [3,4,7-16]

Salm and Sissons [7] first described two cases of extraosseus giant-cell tumors having histologic features identical to bony ABC in 1972. Since then, 16 cases (including this report) have been reported occurring in various locations, and are summarized in Table 1. Patients are made up of eleven females and six males ranging in age from 7 – 60 years with a median age of 28. Tumors showed histologic features identical to the bony ABC and ranged in size from 1.8cm to 10cm with a median of 4cm. Lesions were most often located in the deep soft tissues of the upper extremity or shoulder [7], or thigh. [3] All cases described were treated by surgical excision. Recurrence rate is unknown due to the small number of cases and inadequate follow-up.

Table 1  Summary of reported cases of soft tissue aneurysmal bone cyst (ABC).

Recently, Karkuzhali et al., [15] described a case of multiple soft tissue ABC’s arising following excision of a bony ABC from the proximal fibula 20 months prior, possibly suggesting a neoplastic course. Cytogenetic studies [3,18] have shown an association with translocations at the 16q22 and 17p13 loci, providing further evidence of a possible neoplastic etiology. It has been proposed that the lesion is first formed as a reaction to ectopic bone, but is more likely the result of a primary arteriovenous malformation. [3]

Despite the usual appearance of aneurysmal bone cyst in long bone metaphyses, the foot and ankle surgeon should be aware that it may also manifest as a soft tissue lesion of the lower extremity.

The differential diagnosis of soft tissue ABC can include nodular fasciitis and ossifying fibromyxoid tumor. Nodular fasciitis lesions may histologically mimic soft tissue ABC, but do not contain the characteristic pseudocystic spaces or thin shell of bone. Ossifying fibromyxoid tumors often show spicules of bone, but are without osteoclastic-like giant cells characteristic of soft tissue ABC. [3] Definitive diagnosis is made based on microscopic pathology, which is undistinguishable from the classic bony lesion. (Fig. 4)

Figure 4  Pathologic identification of soft tissue ABC.

The exact etiology of soft tissue ABC’s remains unknown. While the lesion can be aggressive in growth, it is benign and can be successfully treated with surgical excision.

References

1. Vergel De Dios AM, Bond JR, Shives TC, McLeod RA, Unni KK. Aneurysmal bone cyst. A clinicopathologic study of 238 cases. Cancer 1992 69: 2921-2931.
2. Leithner A, Machacek F, Haas OA, Lang S, Ritschl P, Radl R, Windhager R. Aneurysmal bone cyst: a hereditary disease? J Pediatr Orthop B 2004 13: 214-217.
3. Nielsen GP, Fletcher CD, Smith MA, Rybak L, Rosenberg AE. Soft tissue aneurysmal bone cyst: a clinicopathologic study of five cases. Am J Surg Pathol 2002 26: 64-69.
4. Fellig Y, Oliveira AM, Margolin E, Gomori JM, Erickson-Johnson MR, Chou MM, Umansky F, Soffer D. Extraosseous aneurysmal bone cyst of cerebello-pontine angle with USP6 rearrangement. Acta Neuropathol 2009 118: 579-581.
5. Abuhassan FO, Shannak AO. Subperiosteal resection of aneurysmal bone cysts of the distal fibula. JBJS 2009 91B:1227-1231.
6. Chowdhry M, Chandrasekar CR, Mohammed R, Grimer RJ. Curettage of aneurysmal bone cysts of the feet. Foot Ankle Int 2010 31: 131-135.
7. Salm R, Sissons HA. Giant-cell tumors of soft tissues. J Pathology 1972 107: 27-39.
8. Amir G, Mogle P, Sucher E. Case report 729: myositis ossificans and aneurysmal bone cyst. Skeletal Radiology 1992 21: 257-259.
9. Petrik PK, FindlayJM, Sherlock RA. Aneurysmal cyst, bone type, primary in an artery. Am J Surg Pathol 1993 17: 1062-1066.
10. Rodriguez-Perralto JL, Lopez-Barea F, Sanchez-Herrera S, Atienza M. Primary aneurysmal cyst of soft tissues (extraosseus aneurysmal cyst). Am J Surg Pathol 1994 18:632-636.
11. Lopez-Barea F, Rodriguez-Perralto JL, Burgos-Lizaldez E, Alvarez-Linera J, Sánchez-Herrera S. Primary aneurysmal cyst of soft tissue: report of a case with ultrastructural and MRI studies. Virchows Arch 1996 428: 125-129.
12. Shannon P, Bedard Y, Bell R, Kandel R. Aneurysmal cyst of soft tissue: report of a case with serial MRI and biopsy. Human Pathol 1997 28: 255-257.
13. Ajilogha KA, Kaur H, Duncan R, McFarlane JH, Watt AJ. Extraosseous aneurismal bone cyst in a 12 year-old girl. Pediatr Radiol 2005(12):1240-2.
14. D’Costa GF, Hastak MS, Patil YV. Primary aneurysmal cyst: bone type in the breast. Ind J Surg 2007 69: 248-250.
15. Karkuzhali P, Bhattacharyya M, Sumitha P. Multiple soft tissue aneurysmal cysts: An occurrence after resection of primary aneurysmal bone cyst of fibula. Ind J Orthop 2007 41:246-249.
16. Sahu A, Gujral SS, Gaur S. Extraosseous aneurysmal cyst in hand: a case report. Cases J 2008 1: 268.
17. Harrop JS, Schmidt MH, Boriani S, Shaffrey CI. Aggressive “benign” primary spine neoplasms: osteoblastoma, aneurysmal bone cyst, and giant cell tumor. Spine 2009 34 (22 Suppl): S39-47.
18. Dai cin P, Kozakewich HP, Goumnerova L, Mankin HJ, Rosenberg AE, Fletcher JA. Variant translocations involving 16q22 and 17p13 in solid variant and extra osseous forms of aneurysmal bone cyst. Gen Chrom Cancer 2000 (28): 233-234.

Address correspondence to: Kevin McCann, DPM. Franciscan Foot and Ankle Institute, 34509 9th Ave S. Ste 306 Federal Way WA 98003
kevinmccann@fhshealth.org

1,2  PGY 2. Franciscan Foot and Ankle Institute, 34509 9th Ave S. Ste 306 Federal Way WA 98003.
3 Attending Staff, Franciscan Foot and Ankle Institute, 34509 9th Ave S. Ste 306 Federal Way WA 98003.

© The Foot and Ankle Online Journal, 2011

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Lymphangioma of the Foot: A case report

Posted on February 1, 2011 | Comments Off on Lymphangioma of the Foot: A case report

by Steven F. Boc, DPM,FACFAS,FACFAOM 1 , Panagiotis Panagakos, DPM 2 , Soorena Sadri, DPM 3 

The Foot and Ankle Online Journal 4 (2): 1

Vascular Malformations (VM) are localized or diffuse errors of embryonic development that may affect any segment of the vascular tree, including arterial, venous, capillary and lymphatic vessels. Lymphangiomas are rare vascular lymphatic malformations (LM). The rate of occurrence in the foot and ankle regions has been scarcely reported. Lymphangiomas are slow flow vascular malformations and are histological classified into capillary, cavernous and cystic. Treatment options for lymphangiomas include schlerotherapy, the use of angiogenesis inhibitors; however complete surgical resection is the recommended treatment.

Key words: Lymphangioma, tumor, lymphatic malformations, vascular malformation, soft tissue mass, hemangioma, foot, vascular anomaly.

Accepted: January, 2011
Published: February, 2011

ISSN 1941-6806
doi: 10.3827/faoj.2011.0402.0001

Lymphangiomas are rare soft tissue vascular malformations that can appear on the body. [1,2] Lymphangiomas of the foot and ankle is a rare occurrence.[3] Lymphangiomas are equally distributed among genders and races. In the literature, their incidence in children is estimated to amount to 6% of all benign tumors. In about half of the patients the disease is already obvious at the time of birth. The tumor commonly appears as a ballotable mass and the overlying skin is usually normal, but may have a bluish hue. Lymphangiomas of the extremity can cause diffuse swelling or localized swelling or gigantism with soft tissue and skeletal overgrowth. They are commonly misdiagnosed as Hemangiomas. [1,4,5,6]

Vascular malformations (VM) are localized or diffuse errors of embryonic development that may affect any segment of the vascular tree, including arterial, venous, capillary and lymphatic vessels. During Embryogenesis the development of the vascular system occurs by two separate but related processes; vasculogenesis and angiogenesis. Development of the lymphatic system begins in the sixth to seventh week of gestation, approximately four weeks after the onset of vasculogenesis. Existing veins give rise to lymph sacs which then bud lymphatic capillaries in a centrifugal manner. There is an in situ differentiation of lymphangioblasts from mesenchymal cells into lymphatic endothelial cells with subsequent recruitment of these cells into developing lymphatic vessels.

Vasculogenesis, angiogenesis and lymphangiogenesis are subject to precise regulation of growth factors, intercellular and extracellular matrix signaling molecules including vascular endothelial growth factor (VEGF) and the VEGF receptor families, angiopoietins and the Tie-2 receptor, transforming growth factor-β and its receptor, PDGF-B and its receptor, the Notch and Jagged families of membrane associated molecules and the integrin family of cell surface receptors. The integrins which mediate interactions within the extracellular matrix are important in the formation of the vascular and lymphatic systems. Mutations or deletions in specific integrin subtypes can lead to abnormal lymphatic development. [4,5] In 2007 Skandalakis, et al., described the formation of the lymphatic system which they clearly stated is an enigma. They report the formation of a lymphangiomas as a very rare phenomenon. [2]

The greatest impediment for the diagnosis and treatment of vascular anomalies has been the confusing terminology. [5] A biologic classification system introduced in 1982 based on studies correlating physical findings, natural history and cellular features has clarified most of the terminologic disorder. There are two major types of vascular anomalies: tumors and malformations. Vascular tumors are endothelial neoplasms characterized by increased cellular proliferation. Hemangioma is the most common and is almost exclusive to infants. Vascular malformations are the result of abnormal development of vascular elements during embryogenesis and fetal life. These may be single forms (capillary, arterial, lymphatic or venous) or a combination. Vascular malformations do not generally demonstrate increased endothelial turnover. They are designated according to the predominant channel type as capillary malformations, lymphatic malformations (LMs), venous malformations (VMs), arteriovenous malformations, and complex forms such as capillary-lymphatico-venous malformations. Malformations with an arterial component are fast-flow while the remainders are slow-flow. [4,5,6]

Case Report

A 25 year-old female presented to our office in April of 2009 with complaints of multiple discolored and enlarged painful masses on the plantar aspect of her left foot. (Figs 1A, 1B, and 1C) The masses have been present since early childhood but recently have caused her pain while ambulating. Radiographs were obtained upon initial presentation but were unremarkable. Aspiration of the masses was attempted in the office. The aspirate contained dark red fluid which was sent for culture, which came back negative for any infective process. It was decided at this point to send the patient for a series of diagnostic exams. Bilateral lower extremity venous ultrasound resulted in no evidence of a deep vein thrombosis (DVT). Bilateral ankle brachial indexes were unremarkable as well.

Figure 1A, 1B and 1C Initial presentation of lymphangioma on plantar aspect of foot.(A),  A more oblique view of the lesion. (B). A more lateral view of the lesions. (C)

Magnetic resonance imaging (MRI) was performed for the left foot which showed an enhancing soft tissue mass involving the quadrates plantae, flexor digitorum brevis and flexor hallucis muscle bellies consistent with a hemangioma. (Figs. 2A, 2B and 2C) Due to the extensive nature of the soft tissue masses and severe pain it was determined that surgical excision be performed. The patient received a popliteal nerve block pre operatively for pain control. One plantar incision was used to access the most painful soft tissue masses. It was noted that once the ankle tourniquet was inflated the masses decreased in size.

Figure 2A, 2B and 2C MRI of lymphangioma; sagittal view (A), Axial view (B) and Coronal view. (C)

The masses were excised and sent to pathology for evaluation. (Figs. 3A, 3B and 3C) The final report by the pathologist was dense fibrocollagenous tissue, nerve bundles and few dilated endothelial lined spaces and enlarged lymphatic sacs suggestive of a lymphangioma. (Figs. 4A and 4B)

Figure 3A, 3B and 3C Intra operative removal of the lymphangioma (A and B).  Removed lymphangioma on the back table. (C)

Figure 4A and 4B 10x hematoxylin & eosin slide, shows the presence of many dilated vascular channels lined by flattened endothelial cells and containing lymphatic fluid. (A)  The 40x hematoxylin & eosin slide, shows the presence of many dilated vascular channels lined by flattened endothelial cells and containing lymphatic fluid.

The patient returned the next day to the emergency department in excruciating pain and unable to bear weight on the left foot. There were no signs of infection present at this time (Fig. 5A). Patient was admitted for pain management and was discharged 4 days later when her pain was controlled by simple oral analgesics. Patient presented to the office three weeks after excision with a dehiscence of the surgical site. She was admitted at this point and an incision and drainage with primary closure was performed during this hospital stay (Fig. 5A and 5B). She returned approximately 2 weeks after her second admission to our office.

Figure 5A and 5B One day excision of the lymphangioma. (A)  The patient returned to the operating suite for operative incision and drainage of additional serous fluid with primary closure.(B)

At this point there were no signs of infection and the sutures were removed. (Figs. 6A and 6B) The surgical site appeared healed and no evidence of recurrence of the lymphangioma was apparent at this point. The patient was lost to follow up after this office visit.

Figure 6A and 6B 2 weeks following the Incision and drainage and foot appears to be healing.  (A and B)

Discussion and Conclusion

Lymphangiomas are usually noted at birth or within 2 years of life. [1] Prenatal ultrasonography can detect relatively large lesions as early as the second trimester, although lymphangiomas are frequently misdiagnosed as other pathologic entities. Lymphangiomas most commonly occur in the cervicofacial region, axilla/chest, mediastinum, retroperitoneum, buttock and perineum.

The tumors most commonly appear as ballotable masses the overlying skin is usually normal, but may have a bluish hue. Less common dermal involvement manifests as puckering or deep cutaneous dimpling. Lymphangiomas in the subcutis or submucosa manifest as tiny vesicles. Intravascular bleeding is evident by tiny, dark red domed shaped nodules. [5]

Radiologic documentation is best performed by MRI. Ultrasound is a useful auxiliary agent to confirm the presence of macrocystic lymphangiomas. Lymphangiomas, like hemangiomas and most vascular malformations, demonstrate hyperintense signal intensity in T2 weighted and turbo STIR images. Lymphangiomas demonstrate rim enhancement after contrast application. Microcystic lesions have an intermediate signal on T1 sequences and an intermediate to high signal on T2 sequences. Macrocystic lesions show low intensity in T1 and high intensity in T2. Conventional contrast lymphangiography is rarely performed. [1,4,7]

Histologically, lymphangiomas consist of ecstatic lymphatic channels, occasionally containing a mild lymphocytic infiltrate. Classification by histologic appearance includes three subtyes: capillary, cavernous and cystic. The capillary form is composed of small thinned walled lymphatic vessels. Cavernous consists of large lymphatic channels with adventitial coats.

The third and most common type, cystic lymphangioma is composed of macroscopic lymphatic spaces. These lesions are often multiseptated, which is suggestive of infiltration across the tissue planes. [1,3]

Lymphangioma rarely affects the foot and ankle region. Wu in 1996 described a case of a 12 year-old female with a lymphangioma of the anterior aspect of her ankle. He promotes the use of Lymphangiography for diagnosis and recommends for complete surgical excision for treatment. They usually appear in the cervicofacial area up to 75% of the time. [3] In 2004, Ly, et al., described another case of lymphangioma in a 2 year-old girl’s foot. They recommend Doppler sonography of the mass which reveals no remarkable blood flow. They further describe how to differentiate a lymphangioma from a hemangioma. On MRI, lymphangiomas are characterized by the absence of feeding vessels and lack of intense contrast enhancement. Because of the infiltrating nature of the lesion, complete excision rates are only 18-50%. [1] It appears that there have not been a reported percentage of lymphangioma occurrences of the foot and ankle region. In 2009, Itakura, et al., reported 114 cases of lymphangiomas, which of only 2 occurred in the foot. According to their findings the incidence of lymphangioma of the foot is 1.75%. [8]

A purely osseous lymphangioma is a very rare lesion. Bickel and Borders first described lymphangioma of bone in 1947. They described a case of a 5 year girl with an intraosseous lymphangioma of her Ilium. They speculated that as to whether this tumor could have risen in the soft tissue surrounding the bone and caused secondary erosion of the ilium. [9] In 1977, Jumbelic described the 5th reported case in bone, which was located in the medullary cavity of a long bone, the humerus of a 3 year-old girl. [10] In 1968, Rosenquist and Wolfe reported one of the first rare cases of an intraosseous lymphangioma of a lower extremity bone. They described a 3 year-old male with lymphangioma of his femur, tibia and fibula.

Although lymphangiomas are mainly a soft tissue pathology they can occur within bone, but remain extremely rare in the lower extremities. [11]

The two main complications of that can arise from lymphangiomas are intralesional bleeding and infection. Lymphangiomas often swell in the event of a viral or bacterial infection. Most often this is a harmless event likely related to change in flow or alterations. Bacterial cellulitis, however, is more dangerous and requires prolonged intravenous antibiotics. The two main strategies used to treat lymphatic anomalies are schlerotherapy and surgical resection. Schlerotherapy works through obliteration of the lymphatic lumen. Macrocystic lymphangiomas is more likely than microcystic tissue to shrink after an injection of sclerosant. Ethanol is considered to be the most effective sclerosing agent for low-flow malformations, success rates have been reported between 20% to 65%. Intralesional bleomycin has a reported success of approximately 80% with lymphangiomas of the head and neck. OK-432, a lyophilized mixture of attenuated group A Streptococcus pyogenes of human origin, has also been reported to have dramatic results reported. Resection is the only way to potentially eliminate the lymphangioma. Complete resection is the operative goal. Resection should be staged and the surgeon should focus on a defined anatomic region. Postoperative wound complications are common, including drainage, seroma and infection. A compressive dressing should be applied immediately post operatively to prevent complications. Even with an intensive approach to resection, the recurrence rate is reported to be 40% after an incomplete excision and 17% after a macroscopically complete excision. Angiogenesis inhibitors are at the forefront of medical research for the treatment of venous malformations and neoplasms. Blocking new blood vessel formation may limit the ability of the malformation or neoplasm to fully develop. Approximately 20 angiogenesis inhibitors are being tested in human trials. Research for the development of new therapeutic strategies for vascular tumors by inhibiting angiogenesis is underway. [4,5,6]

In conclusion, lymphangiomas are rare in the foot and ankle region. A thorough history and physical are necessary to help distinguish from other benign soft tissue tumors. [5] Imaging studies such as radiographs and MRI are highly recommended to aid in the diagnosis of these tumors. Surgical resection of the mass is recommended for the treatment with the goal of complete excision. The specimen should be sent to pathology for confirmation of the diagnosis. Surgical resection was recommended for this patient. Overall, the patient had minor post operative complications with no sequela. Our recommendation on the day of surgical intervention is to administer a proximal regional block in the popliteal fossa for a longer duration of pain relief post operatively and to admit the patient for observation and pain management. Infection is a common complication for this procedure and the patient should receive pre operative antibiotics and possibly continue them for one week post resection to avoid this complication. The patient was followed for approximately 6 weeks post resection of the tumor and was doing satisfactory, no pain was present upon stance or ambulation. Unfortunately the patient was lost to follow-up due to her relocation to another country.

References

1. Ly Q, Gilbert B, Davis S, Beall D, Richardson R. Lymphangioma of the foot. Am J Roent 2005 184:205-206.
2. Skandalakis J, Skandalakis L, Skandalakis P. Anatomy of the lymphatics. Surg Oncol Clin N Am. 2007 16:1-16
3. Wu K. Lymphangioma of the ankle region. J Foot Ankle Surg 1996 35(3):263-65
4. Christison-Lagay E, Fishman S. Vascular anomalies. Surg Clin N Am 2006 86:393-425.
5. Marler J, Mulliken J. Current management of hemangiomas and vascular malformations. Clin Plastic Surg 2005 32:99-116.
6. Dohil M, Baugh W, Eichenfield L. Vascular and pigmented birthmarks. Ped Clin N Am 2000 47(4):783-812.
7. Damron T, Beauchamp C, Rougraff B, Ward W. Soft-tissue lumps and bumps. JBJS 2003 85A:1142-55.
8. Itakura E, Yamamoto H, Oda Y, Furue M, Tsuneyoshi M. VEGF-C and VEGFR-3 in a series of lymphangiomas: is superficial lymphangioma a true lymphangioma? Virchows Arch 2009 454(3):317-259.
9. Bickel W, Broders A. Primary lymphangioma of the ilium: Report of a case. JBJS1947 29A:517-22.
10. Jumbelic M, Feuerstein IM, Dorfman HD. Solitary intraosseous lymphangioma: A case report. JBJS 1984 66A:1479-481
11. Rosenquist C, Wolfe D. Lymphangioma of bone. JBJS1968 50A:158-6.

Address correspondence to: Steven F. Boc, DPM, FACFAS, FACFAOM, 235 North Broad Street, Philadelphia, PA 19107.

1 Director of Podiatric Medicine and Surgery Residency Program, Hahnemann University Hospital/Associate Professor Department of Surgery Drexel College of Medicine.
2 PGY3 Podiatric Surgery and Medicine Resident, Hahnemann University Hospital/Drexel College of Medicine.
3 PGY2 Podiatric Surgery and Medicine Resident, Hahnemann University Hospital/Drexel College of Medicine.

© The Foot and Ankle Online Journal, 2011

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Leiomyosarcoma of the Foot: A case report

Posted on November 1, 2010 | Comments Off on Leiomyosarcoma of the Foot: A case report

by Sutpal Singh, DPM, FACFAS1, Chih-Hui (Jimmy) Tsai, DPM2, Albert Kim, DPM3, Timothy Dailey, DPM4, Vir Nanda, MD5,
Rao Paladugu, MD6

The Foot and Ankle Online Journal 3 (11): 1

The authors present a case report of a rare leiomyosarcoma. The tumor presented as an indurated mass on the dorsum of the foot. The mass was excised, and further treated by an oncologist. It is important to be suspicious of any large tumor on the dorsum of the foot because of possible malignancies. Early diagnosis and treatment contribute to a better prognosis.

Key Words: Leiomyosarcoma, tumor of the foot, smooth muscle cell tumor.

Accepted: October, 2010
Published: November, 2010

ISSN 1941-6806
doi: 10.3827/faoj.2010.0311.0001

Leiomyosarcoma has been recognized for many years. Leiomyosarcomas can be divided into three types based on their site of origin. The most common type is leiomyosarcomas of soft tissues. The best prognosis is cutaneous leiomyosarcoma and arises from the small muscles in the hair follicles. The rarest type is vascular leiomyosarcomas. [1] It is a type of spindle cell sarcoma, which is a soft tissue tumor, originating from smooth muscle. These tumors most commonly affect the uterus and the abdominal cavity. [2] It occurs in the fifth to seventh decade and affects more males than females. [3] Leiomyosarcoma in the foot is relatively rare and infrequently reported. Only 21 cases of leiomyosarcoma in both the foot and ankle have been reported in the literature since 1936. [4]

Approximately 350 spindle cell sarcomas are diagnosed each year and of these only eight percent are leiomyosarcomas. Of these eight percent, only about four percent occur in the foot and ankle. [5] This approximately equates to 1 case per year. These tumors can present as a non-painful mass which grows larger than normal, can be single nodes or multi nodular. Due to the non-painful nature of this tumor, during its early stages, diagnosis can be greatly delayed. Treatment for subcutaneous leiomyosarcoma is resection and some authors suggest postoperative radiotherapy. [1]

The strongest prognostic factor for survival is the presence of metastasis at diagnosis, with the most common site being the lungs. The prognosis for patients with purely cutaneous leiomyosarcoma is an unusually good 90% survival. [4] The patient should then be rescanned every 3 months for the first 2 years and then every 6 months for the next 3 years. [4] In a case paper by Rifleman, Cronin, and Sage, they reported a recurrence rate of approximately 60%. [6]

Here, we report a case of subcutaneous leiomyosarcoma, which was initially diagnosed as a ganglionic cyst and was referred to podiatry by a primary care physician for evaluation. As it turned out, the mass was not a cyst, but a sarcoma.

Case Report

This is a 33-year-old female who presented to the office with soft tissue swelling for many years. (Fig. 1) It is now a large mass on the dorsum of the right foot and is painful. Her primary care physician had diagnosed it as a ganglion cyst and the patient was referred for an aspiration, which yielded no fluid. The needle aspirate was then sent for tissue analysis. The pathology report indicated that the aspirate cells were not typical of a benign ganglion .(Fig. 2) An open biopsy was scheduled. An magnetic resonance image (MRI) was ordered before biopsy. The MRI showed that there was a well-circumscribed soft tissue mass measuring 3.2 cm in length and 2.2 cm in thickness. There was also a fairly uniform signal with increased signal compatible with the soft tissue mass with no destruction of the underlying bones or extension into the deep tissue. (Figs. 3A and 3B)

Figure 1  Preoperative view of the foot with a distinctive dorsal mass along the top of the foot.

Figure 2   Aspiration biopsy:  Labeled soft tissue right foot showing scattered atypical cells.

 

Figure 3A and 3B  MRI:  Note the soft tissue mass dorsal to the underlying bones.  Coronal section (A) and lateral view shows lesion directly over midtarsal joint region. (B)

The computed tomography (CT) scan also confirmed a similar sized mass located subcutaneously along the dorsal aspect of the tarsal bones with uniform CT density, no irregularity, and no extension of the mass lesion or infiltration or destruction of the underlying bones (Fig 4).

Figure 4   CT scan: Note that the soft tissue mass has not infiltrated the underlying tarsal bones.

Surgical Techniques

The patient had an open biopsy performed with a small incision overlying the mass down to the subcutaneous level. Several 2 mm open punch biopsies were taken. The pathology report yielded a high-grade mesenchymal neoplasm consistent with sarcoma. (Fig. 5) The patient had a wide excisional resection of the mass performed at a later date. The patient was placed in a supine position and then a # 10 blade was used to make an incision over the mass. This was deepened to the subcutaneous tissue. (Figs. 6A and 6B) The mass was noted under and engulfing the extensor hallucis longus tendon. The tissue was then dissected to the deeper levels just superior to the first three rays and corresponding cuneiforms.

Figure 5   Soft tissue open incisional biopsy: High grade neoplasm consistent with considerable pleomorphism of tumor cells.

 

Figure 6A and 6B  Soft tissue mass being resected.  Note that the extensor hallucis longus is completely infiltrated by the mass. (A)  The unencapsulated tumor shows diffuse infiltration of adjacent structures. (B)

The entire mass, the involved extensor hallucis longus tendon, and surrounding tissue were all removed and sent for pathological analysis. The area was then copiously irrigated with normal saline and bacitracin and then closed using 3-0 Vicryl and 3-0 Prolene.

Results

The tumor presented a gray white cut surface and it was unencapsulated. The tumor consisted of diffuse sheets of elongated spindle shape cells with moderate amounts of eosinophilic cytoplasm and elongated nuclei which are hyperchromatic with rounded ends. The neoplastic cells are also arranged in fascicles with the nuclei showing considerable pleomorphism. The mitosis was variable and average 8 per 10 high power fields. Multinucleated tumor giant cells are readily seen. Mitosis is helpful in differentiating these tumors from their benign counterparts. In view of the location of the tumor, secondary changes like pressure necrosis, inflammatory changes due to trauma and focal hemorrhage are also noted. Other areas of the resected tumor show fibrocollagenous adipose, muscle and tendinous tissue with focal areas of infiltration by the malignant tumor cells. Special staining for actin, myoglobin, S-100 protein, vimentin, and CD-57 were also performed. The actin, myoglobin and vimentin were positive, the S-100 was weakly positive and the CD-57 was negative. (Fig. 7) In light of this immunohistochemical and microscopic analysis, a diagnosis of poorly differentiated malignant tumor with features consistent with leiomyosarcoma was made.

Figure 7  Histopathological findings of the excisional biopsy.  Note the tumor giant cell in the middle photograph.

Discussion

Leiomyosarcomas are rare, resistant, malignant soft tissue tumors that originate and mutate from involuntary smooth muscle cells. In this case report, the most likely category of leiomyosarcoma would either be cutaneous or subcutaneous.

Cutaneous leiomyosarcomas are derived from precursor cells associated with the arrector muscles of the hair. Cutaneous leiomyosarcomas often present as a solitary nodule smaller than 2 cm, usually on the extremities, particularly on the hair bearing extensor surfaces and on the lower extremities. The epidermis over the lesion may exhibit a pink, purple, brown, or red discoloration with possible crusting or ulceration. Although a minority of patients are asymptomatic, a majority of patients report pain. [1]

Subcutaneous leiomyosarcomas originate from the muscular walls of veins and arteries. The inferior vena cava and veins of the lower extremities are the most common sites. This type of leiomyosarcoma has a more rapid growth rate than cutaneous leiomyosarcomas.

Pain and numbness are the most common symptoms in contrast to cutaneous leiomyosarcomas, these lesions may appear well circumscribed. The risk of metastasis is greater than in cases of cutaneous leiomyosarcomas and the risk appears to increase with depth. [9,10]

In this case, the lesion was presumed to be a ganglion cyst from the shape, location, and clinical presentation. The advanced imagining also presented the lesion to be a ganglion cyst because of signal intensities on MRI, as well as the well circumscribed nature.

However, this was not the case after aspiration and several biopsies suggesting a different diagnosis. The ultimate diagnosis of leiomyosarcoma was determined after histologic findings and immunohistochemical assays with evidence of neoplastic cells and positive finding for special staining for actin, myoglobin and vimentin, and S-100 protein. [11]

This patient most likely had a subcutaneous leiomyosarcoma due to the well circumscribed nature and deeper location. Although cutaneous leiomyosarcomas are more common on the extensor surfaces of the lower extremities, a cutaneous leiomyosarcoma was ruled out due to the lack of epidermal changes, the larger size of this lesion, and the deeper location. Interestingly, the pathology report noted marked pleomorphism that may categorize this lesion to be pleomorphic leiomyosarcoma, which is a morphologic variant. [13]

The treatment of leiomyosarcomas is directed by its stage, location, size, and patient’s age. Since this leiomyosarcoma was well circumscribed and the ability to take a wide excisional resection of the mass was possible, it was determined that the wide excisional resection was the most appropriate treatment. Note that a below the knee amputation is also a viable choice but the patient refused this option. The patient also received radiation therapy and this seems to improve the local control of the disease. [12] The literature recommends patients should be observed for at least 5 years after the surgery with most recurrence occurring at around the 2 year mark. [13,14]

The early detection of this leiomyosarcoma, wide excision, and the radiation therapy has resulted in a satisfactory outcome in this case. (Fig. 8) However, recurrences can occur and it is recommended to have periodic follow ups.

Figure 8  Three year follow up after radiation treatment.

Conclusion

Though rare in nature, it is important to always be suspicious of a large mass on the dorsal aspect of the foot. Diagnosis of the pathology should be performed in a timely matter. Also, appropriate consultations and radiographic studies should be used in order to examine the extent of involvement.

References

1. Abed R, Abudu A, Grimer J, Tillman M,Carter R, Jeys L. Leiomyosarcomas of vascular origin in the extremity. Sarcoma 2009.
2. Svarvar C, Böhling T, Berlin Ö, Gustafson P, Follerås G, Bjerkehagen B, Domanski HA, Sundby Hall K, Tukiainen E, Blomqvist C. Clinical course of nonvisceral soft tissue leiomyosarcoma in 225 patients from the Scandinavian sarcoma group. Cancer 2007 109(2).
3. Angeloni M, Muratori F, Magarelli N, Chalidis BE, Ricci R, Rossi B, Maccauro G. Exophytic growth of a neglected giant subcutaneous leiomyosarcoma of the lower extremity. A case report. International Seminars in Surgical Oncology 2008 5:11.
4. Erin E, Butler M, Anain Jr J. Leiomyosarcoma of the foot. JAPMA 2007 97(6): 475-479.
5. Kransdorf M. Malignant soft tissue tumors in a large referral population: Distribution of diagnoses by age, sex, and location. American J Radiology 1995 164: 129-134.
6. Rifleman GT, Cronin R, Sage R. Leiomyosarcoma of the Cutaneous Tissue. JAPMA 1990 80(4): 222-225.
7. Efstathopoulos N, Lazarettos J, Vassilios N, Efstathios C. Inflammatory leiomyosarcoma of the ankle: A case report and review of the literature. J Foot & Ankle Surg 2006 45(2):127-130.
8. Bulmer JH. Smooth muscle tumors of the limbs. JBJS 1967 49B(1) 52-58.
9. Fields JP, Helwig EB. Leiomyosarcoma of the skin and subcutaneous tissue. Cancer 1981 47:156-169.
10. Dahl I, Angervall L. Cutaneous and subcutaneous leiomyosarcoma: A clinicopathologic study of 47 patients. Pathologia Europea 1974 9: 307.
11. Suster S. Epithelioid leiomyosarcoma of the skin and subcutaneous tissue: clinicopathologic, immunohistochemical, and ultrastructural study of five cases. Am J Surg Pathol 1994 18: 232-240.
12. Angeloni M, Muratori F, Magarelli N, Chalidis BE, Ricci R, Rossi B, Maccauro G. Exophytic growth of a neglected giant subcutaneous Leiomyosarcoma of the lower extremity. A case report. Int Semin Surg Oncol 2008 May 21(5):11.
13. Tran L, Mark R, Meier R, Calcaterra T, Parker R. Sarcomas of the head and neck. Prognostic factors and treatment strategies. Cancer 1992 70:169–177.
14. Oda Y, Miyajima K, Kawaguchi K, Tamiya S, Oshiro Y, Hachitanda Y, Oya M, Iwamoto Y, Tsuneyoshi M. Pleomorphic leiomyosarcoma: clinicopathologic and immunohistochemical study with special emphasis on its distinction from ordinary leiomyosarcoma and malignant fibrous histiocytoma. Am J Surg Pathol 2001 25(8):1030-1038.

Address correspondence to: Sutpal Singh, DPM, FACFAS, Chief Ilizarov Surgical Instructor at Doctors Hospital West Covina, Fellow of the American College of Foot and Ankle Surgeons, Private practice in Southern California.

1  Chief Ilizarov Surgical Instructor at Doctors Hospital West Covina, Fellow of the American College of Foot and Ankle Surgeons, Private practice in Southern California.
2  Doctor of Podiatric Medicine (R3) ,Foot and Ankle Medicine and Surgery, Doctors Hospital of West Covina (PM&S-36).
3  Doctor of Podiatric Medicine (R2) Foot and Ankle Medicine and Surgery, Doctors Hospital of West Covina (PM&S-36).
4  Doctor of Podiatric Medicine (R1) Foot and Ankle Medicine and Surgery, Doctors Hospital of West Covina (PM&S-36).
5  Oncologist Private Practice in Southern California.
6  Pathologist at St Mary Regional Medical Center.

© The Foot and Ankle Online Journal, 2010

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