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Indian Journal of Surgery, Vol. 66, No. 2, Mar-Apr, 2004, pp. 110-112 Case Report Kaposi's sarcoma in a follow-up patient of malignant schwannoma after seroconversion A. Gopala Krishna, G. V. Ramana Reddy Department of Plastic Surgery, St.Theresa's Hospital, Hyderabad - 500018,
India.
Paper Received: December 2003. Paper Accepted: February 2004. Source of Support: Nil. Code Number: is04028 ABSTRACT A follow-up case of malignant schwannoma of the right hand presented with multiple nodulo-ulcerative lesions confined to the right upper limb, 3 years after surgery. On investigation the patient tested positive for Human Immuno deficiency Virus (HIV); biopsy of the lesions was reported as Kaposi's sarcoma. Key words Kaposi's sarcoma, malignant schwannoma, seroconversion. How to cite this article: Krishna AG, Reddy GVR. Kaposi's sarcoma in a follow-up patient of malignant schwannoma after seroconversion. Indian J Surg 2004;66:110-2. INTRODUCTION Kaposi's sarcoma was first described in 1872 by Moritz Kaposi as a disease seen in elderly men of Mediterranean or Jewish descent. Four different clinical and aetiological entities have been recognised later: 1) Classical Kaposi's sarcoma 2) African non-HIV variety 3) Kaposi's sarcoma occurring in immunosuppressed patients and 4) Kaposi's sarcoma in AIDS. In 1981, initial reports described it in homosexual men with AIDS. But recent publications have reported its incidence in heterosexual males also.1,2 Now HIV is a fast-spreading epidemic in India. In India there are 39.7 million HIV cases as reported by the UNAIDS global HIV / AIDS Report 2002. Currently, the infection rate is estimated to be 0.7 per cent in the adult population (between 1549 years of age).3 Kaposi's sarcoma was one of the first conditions recognised as an opportunistic sequel of the HIV infection and remains the most common AIDS-associated neoplasm. Although all forms of Kaposi's sarcoma are histologically similar there is a wide range in the distribution and clinical manifestations.4 The disease usually presents initially as violaceous skin lesions, but oral, visceral or nodal involvement may precede cutaneous involvement. Biopsy for definitive diagnosis is recommended to distinguish Kaposi's sarcoma from other pigmented skin conditions.5 CASE REPORT In July 2002 a 35-year-old heterosexual right-handed male, presented with asymptomatic nodulo-ulcerative lesions on the right upper limb since one year. He had past history of malignant schwannoma arising from a digital nerve removed twice from the right palm. In October 1997 an ulcerated lesion was removed from the right palm, which was reported as malignant Schwannoma. He presented with recurrent lesion at the same site in December1998. Recurrent malignant schwannoma was considered as a diagnosis. A wide local excision with sural nerve grafting for digital nerve was done. The wide defect in the palm was covered with radial artery island flap. Histopathology of the specimen was reported as malignant schwannoma with tumour-free margins. It healed completely with reasonable recovery of sensations and he got back to work. On examination there were multiple nodulo-ulcerative lesions ranging from subcutaneous nodules to proliferating ulcers. The ulcers were of varying sizes, the biggest measuring 2 cm X 3 cm on the anterior aspect of the forearm 8 cm below the cubital fossa (this was the first lesion to appear). All the ulcers were non-tender with indurated base and raised edges and the floor was covered with brown to black slough. There were multiple, non-tender subcutaneous nodules measuring from 0.5 cm1 cm in size, confined to the right upper limb only. There was a firm, non-tender mobile lymph node in the right axilla measuring 2 cm X 2 cm. He had normal sensations on clinical examination of the hand. General examination was unremarkable except for the wheeze on auscultation of the lungs on both sides but the patient was comfortable while breathing. On investigation he tested positive for HIV-1. (He had tested negative for HIV when he had been operated for malignant schwannoma in 1998). X-ray chest was normal. In view of wheeze on clinical examination and the fact that he had malignant tumour earlier, a CT scan chest was done, which showed multiple, small, thin-walled cystic lesions with multiple tiny nodular lesions in the periphery of both lungs. Multiple biopsies were taken which included most of the nodulo-ulcerative lesions and also the original site of surgery in the palm. The reports suggested there was only fibrous tissue in the specimen from the previous operation site in the palm. All the other lesions showed histology of Kaposi's sarcoma. DISCUSSION The awareness of cutaneous Kaposi's sarcoma as a diagnostic possibility helps in the work-up of nodulo-ulcerative skin lesions. In this case we did not include Kaposi's sarcoma in the initial clinical differential diagnosis. The rarity of isolated limb involvement in Kaposi's sarcoma and the past history of malignant schwannoma of the same limb contributed to the absence of Kaposi's sarcoma as a diagnostic possibility in the work-up of the patient. Our first clinical diagnosis was recurrent malignant schwannoma because of his past history and because the lesions appeared to be along the distribution of cutaneous nerves. Malignant melanoma was considered in the differential diagnosis because of the pigmented nodules, pigmentation in the ulcers and because the distribution of lesions looked like intransit metastases. Kaposi's sarcoma was considered in the differential diagnosis only when he tested positive for HIV-1. To avoid bias in reporting, the specimen had been sent to three different pathologists, all the pathologists reported it independently as Kaposi's sarcoma. Several different treatments have been used for Kaposi's sarcoma including surgical excision, radiation therapy, Highly Active Anti-Retroviral Therapy (HAART) and intralesional chemotherapy. The initial treatment of patients with Kaposi's sarcoma in HIV positive cases is an effective anti-retroviral regimen. If Kaposi's sarcoma does not regress despite a reduction in HIV viral load and an increase in CD4 cell count, alternative treatments may be considered. Localised lesions may be treated with cryotherapy, laser or surgical excision. But in this case, in view of multiple lesions and involvement of lungs this patient has been put on HAART. REFERENCES
© 2004 Indian Journal of Surgery. |
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