Tag Archives: Kaposi’s sarcoma

Pedal Kaposi’s Sarcoma as the first sign of HIV status: A case report

Posted on August 1, 2008 | Comments Off on Pedal Kaposi’s Sarcoma as the first sign of HIV status: A case report

by Sabrina Minhas, DPM 1, Tracey C. Vlahovic, DPM 2

The Foot & Ankle Journal 1 (8): 3

AIDS-Related Kaposi’s sarcoma is an uncommon occurrence of the foot. Two cases of solitary pedal KS are presented of patients who had not been previously diagnosed as HIV positive prior to biopsy. Once histopathology reports returned the diagnosis of Kaposi’s sarcoma, both patients received HIV tests and were found to be positive.

Key words: Kaposi’s sarcoma, AIDS, pyogenic granuloma

This is an Open Access article distributed under the terms of the Creative Commons Attribution License.  It permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. ©The Foot & Ankle Journal (www.faoj.org)

Accepted: June 2008
Published: August 2008

ISSN 1941-6806
doi: 10.3827/faoj.2008.0108.0003

Kaposi’s sarcoma (KS) or “multiple idiopathic hemorrhagic sarcoma” was first described in the 1800’s. [1] Historically, this disease is known to be a rare, slowly spreading tumor confined almost entirely to older European populations. [2] More recently, it has been shown to occur regularly in the African tropics as well as be associated with AIDS. [2] Kaposi’s sarcoma has been found to be the most prevalent neoplastic lesion in patients who have AIDS in the United States. [3] This type occurs more commonly in patients older than fifty and presents as lower extremity lesions.

African KS, the second type, accounts for the most prevalent tumor in parts of Zaire. Even though this form may affect younger patients, it can be more aggressive by invading bone and causing various other infiltrates. The third type, AIDS-Related KS, is more common in homosexual men. [6] In this type, extracutaneous growth is known to occur. The final type involves iatrogenic immunosuppresion which occurs when patients are given various anti-rejection medications following organ transplantation. KS and other tumors have a greater prevalence in these patients. However, once these drugs are discontinued, this form of Kaposi’s sarcoma has been shown to regress.

Four types of Kaposi’s sarcoma exist and are caused by Human Herpesvirus 8 (HHV-8). [2,4,5] The first type, Classic KS, is found to be prevalent in Jewish, Mediterranean, and Eastern European men and seldom seen in females.

The typical KS lesion tends to be fairly widespread, whereas HIV related KS is characteristically limited to the skin and/or mucous membrances. Classic KS lesions tend to involve the oral cavity, lymphatics, lungs, trunk and extremities. [2,5,6] In AIDS-Related KS, lesions on the foot and ankle are an uncommon occurrence; whereas in the Classic form, pedal lesions are more common. [7] In the AIDS-Related patients, skin lesions are considered manifestation of advanced HIV disease and are more commonly seen on the superior aspects of the trunk and extremities. [8] These lesions may appear as dark pink or purple to brown or black. [8,9] In addition, its appearance can vary from plaques to nodules with crust. [2,8,10] Various differential diagnoses exist for pedal Kaposi’s sarcoma; such as pyogenic granuloma, well-differentiated angiosarcoma, amelanotic melanoma, hemangioma, purpura, lesions associated with vascular insufficiency, and ecchymoses. [10,11] Interestingly, Kaposi’s sarcoma lesions arise slowly and may be painless even in long-standing wounds. [9,10] Essentially, most malignancies of the skin are painless. Diagnosis must be made via a skin biopsy of the questionable lesion. In the cases reviewed in this study, a solitary pedal lesion was the only indication of the patients’ disease.

The exact cause of KS is still a mystery, but in AIDS-Related Kaposi’s sarcoma, it has been shown that HHV-8 toxicity of the patient is related to the viral load, the CD4 count, and the HHV-8 serological activity. [4,7] In rare instances, AIDS-Related KS may even be caused by trauma. [9]

Treatments for KS vary from simple excision to chemotherapy and radiation therapy for more extensive cases. [7-10] Since each lesion of Kaposi’s sarcoma evolves independently from the other, a simple excision may not prevent more from forming. [2,7,10] So, roentgen treatments are considered favorable by many to treat and prevent further KS. [13,14] HIV testing must also be performed on patients who have the lesions and have risk factors.

The following case reports show two patients with non-painful pedal lesions as one of the first signs of HIV infection.

Case 1

A 31 year old male without a significant past medical history presents with a painful “blood blister” to the foot. Medication history included oral terbinafine for toenail onychomycosis. The patient initially complained of a painful “blood blister” that wouldn’t disappear on his right foot for three months. The patient denied any trauma or new activities. He tried to “pop” the blister, but was unsuccessful in generating any sero-sanguinous fluid. Upon clinical examination, the lesion was a well-demarcated solitary reddish-purple nodule (1 cm x 1 cm) on the medial aspect of the first metatarsal mid-shaft with no ulceration or signs of infection. (Fig. 1)

Figure 1  HIV Patient with pigmented lesion on medial aspect of foot.

It was freely mobile and appeared to be a superficial lesion. Differential diagnoses include melanoma, hemangioma, and vascular malformation. The patient requested removal by excision due to the constant rubbing of the lesion against the shoes.

An excisional biopsy was performed in the office. The pathology report returned as Kaposi’s sarcoma which was strongly reactive to CD34 and negative for Smooth Muscle Antigen, Factor XIIIa and Desmin.

When the patient returned to the clinic for suture removal, the diagnosis was discussed and an HIV test with a general laboratory work-up was recommended. Subsequently, the patient’s HIV test was positive and the patient was then referred to the appropriate specialist. To this date, no recurrence of the lesion or of any new lesion has occurred on that foot.

Case 2

A 38 year old male presents with a solitary 0.5 cm x 0.5 cm bright red papule on the plantar surface of the right foot. The lesion was painful and sponataneously occured two months prior to presentation. Patient denied trauma to the area. Patient had no significant medical history, no known allergies to medication, and not taking any medications. The patient requested removal of the lesion. Upon examination, the lesion had the appearance of a pyogenic granuloma and was completely excised in the office. (Fig. 2)

Figure 2  HIV with pyogenic granuloma-like lesion of the plantar foot.

The pathology report showed: mitotic figures, proliferation of spindle cells and slit-like vascular channels with red blood cells. The lesion was further stained for HHV-8 and a subsequent report diagnosed the lesion as Kaposi’s sarcoma. During the patient’s follow up visit to remove sutures, the diagnosis was discussed and an HIV test was strongly recommended.

The patient consented to HIV testing which was positive. He was referred to an HIV specialist. To this date, the foot lesion has not recurred.

Discussion

Pedal AIDS-Related KS is a rare occurrence, and even rarer for a patient’s HIV status to first manifest itself as a KS lesion on the foot. Appearance of KS is considered a sign of both advanced disease and opportunistic infection. [6] Young homosexual men with KS are more likely to present with AIDS-Related Kaposi’s sarcoma while older heterosexual men are at higher risk to acquire Classic Kaposi’s sarcoma. [10,11] Exceptions exist in both cases, and a thorough history and physical exam along with laboratory tests should be performed to evaluate each individual especially post-biopsy.

When evaluating pigmented nodules and tumors on the foot, the podiatric physician should be aware of the common locations of KS lesions and include KS as a differential diagnosis along with other benign lesions. Both patients had successful excisional biopsies of their lesions, although in most cases, more aggressive therapy such as chemotherapy or radiation is warranted to eradicate the lesion. These cases demonstrate the importance of evaluating patients thoroughly post-biopsy in order to obtain an accurate diagnosis which will facilitate future treatment.

References

1. Kaposi, M. Idiopathisches multiplex pigment sarcoma der hau ardin. F. Dermatologie (Berlina) 4:269-273, 1872.
2. Elder DE, Elenitsas R, Johnson BL Jr, Murphy GF. Lever’s Histopathology of the Skin. 9th ed. Philadelphia, PA: Lippincott Williams and Wilkins, p 1229, 2005.
3. Anderson LA, Goedert JJ. Tumor markers and treatments for Kaposi sarcoma. AIDS;21:1637-1639, 2007.
4. Chang Y, Cesarman E, Pessin MS, Lee F, Culpepper J, Knowles DM, et al. Identification of herpesvirus-like DNA sequences in AIDS-associated Kaposi’s sarcoma. Science;266:1865-1869, 1994.
5. Martino RJ, Pulse C, Zegarelli DJ. Classic Kaposi’s sarcoma presenting in the oral cavity: a case report and literature review. Columbia Dental Review 1996.
6. Dezube BJ, Groopman JE. AIDS-related Kaposi’s sarcoma: epidemiology and pathogenesis. Hematology/Oncology Clinics of North America;10:1023-1029, 1996.
7. Montes C, Luepschen OM. Kaposi’s sarcoma of the foot in the HIV patient. The Journal of Foot and Ankle Surgery;33:341-345, 1994.
8. Cohen EJ, Cole D, Stewart DM, Weiss G, Kosinski M, Giorgini R. Kaposi’s sarcoma of the lower extremity as the first sign of AIDS. Journal of the American Podiatry Medical Association;80:127-134, 1990.
9. Berkowitz KD, Bonner AC, Makimaa B, Flash JP, Sasken H, Blaise JF. Trauma-induced Kaposi’s sarcoma of the hallux. An unusual case. Journal of the American Podiatric Medical Association;88:500-505, 1998.
10. Berlin SJ, Jurd JA. Kaposi’s sarcoma in the foot. Clinics in Podiatric Medicine and Surgery;9:849-855, 1992.
11. Cangialosi CP, Schnall SJ. Kaposi’s sarcoma of the foot: a case report. Journal of the American Podiatry Association;66:525-527, 1976.
12. Tedeschi R, Enbom M, Bidoli E, Linde A, De Paoli P, Dillner J. Viral load of human herpesvirus 8 in peripheral blood of human immunodeficiency virus- infected patients with kaposi’s sarcoma. Journal of Clinical Microbiology; 39:4269-4273, 2001.
13. Mazzanti JA, Hugar DW. Kaposi’s sarcoma-an overview. The Journal of Foot Surgery;19:71-73, 1980.
14. Levi MJ. Classic Kaposi’s sarcoma. Journal of the Podiatric Medical Association; 95:586-588, 2005.

Address correspondence to: Tracey C. Vlahovic, DPM
Associate Professor, Temple University School of Podiatric Medicine, Philadelphia, Pa. 19107 email: traceyv@tample.edu

1  Second year resident, Roxborough Memorial Hospital, Philadelphia, Pa. 19107.
2  Associate Professor, Temple University School of Podiatric Medicine, Philadelphia, Pa. 19107.

© The Foot & Ankle Journal, 2008

Comments Off on Pedal Kaposi’s Sarcoma as the first sign of HIV status: A case report

Posted in Uncategorized

Tagged , , , ,

Kaposi’s Sarcoma of the Foot: A Case Report

Posted on March 1, 2008 | Comments Off on Kaposi’s Sarcoma of the Foot: A Case Report

by Al Kline, DPM 1

The Foot & Ankle Journal 1 (3): 1

A case of ulcerated, classic Kaposi Sarcoma (KS) of the foot is described. This is a tumor of vascular endothelial origin which most commonly present in men 50-70 years of age. This case report includes presentation and discussion of differential diagnosis, surgical removal, histopathology interpretation, incidence of metastasis and treatment.

Key words: Kaposi’s Sarcoma, Metastasis, Ulcerated KS, Classic KS, Pedunculated KS

This is an Open Access article distributed under the terms of the Creative Commons Attribution License. It permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. ©The Foot & Ankle Journal (www.faoj.org)

Published online: March 1, 2008

ISSN: 1941-6806/08/0103-0001
doi: 10.3827/faoj.2008.0103.0001

Kaposi’s sarcoma (KS) was first described by Hungarian dermatologist Moritz Kaposi in 1872. [1,2] Kaposi first described the lesions as “idiopathic, multiple pigmented sarcoma of the skin”. Kaposi’s sarcoma is a spindle cell tumor of endothelial origin. It is commonly associated with the endothelial lining of blood vessels. In the early 1980’s, KS was commonly associated with the AIDS epidemic. Four types of Kaposi’s sarcoma have been described that include epidemic AIDS related Kaposi’s Sarcoma, Immunocompromised KS, Endemic African KS and Classic KS. The classic KS is the only form not associated with the AIDS epidemic. In the past two decades, with the improvement in AIDS medication and treatment, the incidence of AIDS related KS has decreased by 90%. [1]

Classic KS was first described as most prevalent in elderly Jewish, Mediterranean men between the ages of 50-70 years. [1,3] The ratio of KS in men to women is 15:1. [1] The highest incidence of KS is reported in Sicily.

In the lower extremity, the lesion is most commonly associated with venous stasis and lymphedema. However, lesions on the plantar surface of the foot are rarely associated with edema which complicates this classic description. The lesions are usually violasceous, red to pink in color. Almost all cutaneous lesions are raised, non-pruritic and symmetrical. The histopathology of KS reveals excessive spindle-cell proliferation throughout the endothelium. Human Herpes Virus 8 (HHV-8) has been identified in over 90% of all KS tumors, suggesting a causative role. [2] The incidence of KS of the foot is rarely reported. This report describes a case of ulcerated, classic Kaposi’s sarcoma of the foot is described.

Case Report

A 71 year old male presents to our office on consultation with a “growth” to the bottom of the left foot. (Figs. 1abc)

  

Figures 1abc The Kaposi’s tumor measuring 30mm in diameter.  This circular soft tissue tumor of endothelial origin is macerated from occlusive dressings placed over the lesion.  A smaller lesion is noted along the plantar, medial border of the foot along the first MPJ.

The patient and family report he has had this lesion for a number of years. It recently became more macerated with ‘bleeding’ due to its size and location on the bottom of the foot. The patient has no history of allergies. His medications include Gabapentin, Metformin, Trental, Glipizide, Metaprolol and Lisinopril. He has type 2 diabetes, hypertension and has undergone previous vein stripping. His physical examination reveals a well nourished male with palpable pulses. Minimal edema was noted to his extremities with some pretibial pitting edema.

The patient has been covering the growth with a large, occlusive bandage that caused significant maceration of the lesion. The patient also had a smaller lesion along the medial distal foot. The patient had the lesion on the bottom of the foot for over two years without discomfort. The lesion is macerated and vascular in appearance, pedunculated and attached to a ‘stalk’. (Fig. 2) MRI of the foot revealed a cutaneous soft tissue growth not extending into subcutaneous fat. (Fig. 3) The patient was taken to surgery to remove both lesions by simple excision.

Figure 2 The tumor is attached to a stalk or pedicle.

Figure 3 The pedunculated tumor is located in the acral skin with nodular proliferation of the dermis.

The patient was brought to the operating room and under local, IV sedation, the lesions were elipsed and removed. Simple horizontal mattress sutures were used to close the excision sites. A simple dressing was dispensed and the region healed without complication. To date, there has been no recurrence of the tumors.

Histopathology

The histological characteristic of KS does not vary between the different clinical types, but the progressive nature of the tumor varies. Early lesions show spindle cell proliferation within the interstitium of the upper dermis close to the vascular plexus. [4] Nodular lesions are characteristic of older and more progressive tumors.

The surgical specimens were sent in formalin-filled containers. Microscopic sections and step-sections show a biopsy of acral skin and ulceration. In the dermis, there is a nodular dermal proliferation of atypical spindle cells arranged in nests and fascicles. (Fig. 4) Within the spindle cell proliferation, there are slit-like vascular spaces with extravasation of red blood cells. (Fig. 5)

Figure 4 Under simple H&E (Hematoxylin–eosin) stain, magnification x 20.  Lesional cells stained positive with CD31 and focally with CD34.

Figure 5 High power view of lesional cells.  Note extravasation of red blood cells in slit-like spaces, magnification x 400.

Slit-like vascular spaces containing erythrocytes are typical of the histologic characteristics of KS. These split-like spaces with extravasation sporadically display features of lymphangioma. [4] Immunoperoxidase stains consisting of HHV-8 and S-100 were performed at the Baylor College of Medicine reference laboratory. This report confirms atypical spindle cells demonstrating nuclear positivity for HHV-8 and negative for S-100. This renders the final diagnosis as ulcerative Kaposi’s sarcoma.

Discussion

Our case describes an isolated, pedunculated and ulcerated classic Kaposi’s sarcoma of the foot with a smaller secondary lesion. The ulcerated type of KS appears to be rare with infrequent reports in the literature. The etiology of this type of lesion is unknown. The tumor has been associated with venous stasis and lymphedema. Our patient had signs of venous congestion and lymphedema, but the tumors location is atypical of this association. In recent studies, the tumor is now associated with human herpesvirus- 8 (HVV-8) or commonly called Kasposi sarcoma herpesvirus (KSHV). [2,4]

It is one of the few viruses now proven to be associated with tumorigenesis in humans. [4] The lesions also appear to be associated with immunosuppression. It could be theorized that classic KS arises in elderly men from some form of immunosuppression or compromise. For example, patients who undergo organ transplants and take rejection drugs to suppress the immune system are 150-200 times more likely to ‘acquire’ Kaposi’s sarcoma. [1]

Commonly, the cutaneous lesions of KS are more diffuse and violasceous in color and distribution. However, the clinical presentation can vary greatly. The differential diagnosis should include acroangiodermatitis, port wine stains of the extremities, Sturge Weber Syndrome, nodular malignant melanoma, angiosarcoma, hemangioma, dermatofibroma, pyogenic granuloma or purpura. [1,2,3]

Treatment of classic KS can vary from simple surgical removal of the lesions to radiation treatment. If lesions are solitary, as in this case, surgical excision of the lesion is the best form of treatment. Brenner, et al, reported 52 patients who underwent primary excision for solitary lesions, 56% had no recurrence of lesions in a median of 15 months. [8] If the classic KS is diffuse, irradiation of water while the legs are submerged in a water tank appears to be an accepted treatment. [3] However, recurrence of lesions seems to be more common when the lesions are diffuse. Isolated lesions are best treated by excision, laser and surgical cauterization. Intralesional interferon injection is still considered controversial. [4] Cryotherapy can be used for superficial lesions but are likely to recur. [3]

In one report, cyrotherapy has been shown to be 70% effective for superficial lesions. [4] Occasionally, the lesions and disease may regress spontaneously. [7]

Incidence of metastasis varies in the literature. Some report that classic KS rarely metastasize. [1] Another report suggests that up to one-third of patients with classic KS develop a secondary primary malignancy, most often non-Hodgkins lymphoma. [3] Other reports suggest that over 35% of patients with the disease of more than 15 years may develop secondary malignancies such as leukemia, myeloma and lymphomas. [4,5,6] It appears that cases should be handled individually and risk should be assessed by the number of lesions and their location. Classic KS rarely disseminate and cause visceral or mucous membrane tumors. Dissemination is seen in less than 10% of the patients with KS. [4] Certainly, if widespread visceral involvement is diagnosed, combination treatment including Chemotherapy, radiation treatment, surgical excision of lesions and conservative care is recommended. [4]

This report describes an unusual case of ulcerated Kaposi’s sarcoma of the foot. Simple excision of the lesion was recommended without further cutaneous or systemic treatment. A smaller lesion on the foot was also removed which was consistent with KS. The surgeon has a variety of options for treatment of such lesions. In this case, surgical excision was the procedure of choice and the region healed without complication. (Fig. 6)

Figure 6   A few weeks after excision of tumor.

Inspection of the patients mucous membranes revealed no other suspected, discolored or violasceous plaques. We have recommended the patient undergo routine colonoscopy to rule out any visceral involvement.

References:

1. American Cancer Society: What is Kaposi Sarcoma, [online], 2008.
2. Fishman, A., Sparano, D.: Kaposi Sarcoma. eMedicine [online], 2008.
3. Dubilier, L.D., Joffe, E.: Case report: Classic Kaposi’s sarcoma, JAAPA, 18 (7), July, 2005. [PDF]
4. Phillips, T.J.: Kaposi’s Sarcoma,Wounds 13 (6): 237-240, 2001. [Online]
5. Piette WW. The incidence of second malignancies in subsets of Kaposi’s sarcoma. J Am Acad Dermatol :16:855, 1987.
6. Safai B, Goo RA. Kaposi’s sarcoma: A review and recent developments. Cancer: 44:419-33. 1981.
7. Chang Y, Cesarman E, Pessin MS, et al. Identification of herpesvirus-like DNA sequences in AIDS-associated Kaposi’s sarcoma. Science 266: 1865-1869, 1994.
8. Brenner B, Rakowsky E, Katz A, Gutman H, et al. Tailoring treatment for classical Kaposi’s sarcoma: Comprehensive clinical guidelines. Int j Oncol: 14 (6), 1097-1102, 1999.

Address correspondence to: Dr. Al Kline, DPM, 3130 South Alameda, Corpus Christi, Texas 78404. Email: Al@Kline.net 

1Adjunct Clinical Faculty, Barry University School of Podiatric Medicine. Private practice, Chief of Podiatry, Doctors Regional Medical Center. Corpus Christi, Texas, 78411.

© The Foot & Ankle Journal, 2008

[Download PDF File for Printing]

Comments Off on Kaposi’s Sarcoma of the Foot: A Case Report

Posted in Uncategorized

Tagged , , , , ,