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Indian Journal of Surgery
Medknow Publications on behalf of Association of Surgeons of India
ISSN: 0972-2068
Vol. 66, Num. 2, 2004, pp. 110-112
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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.
Address for
correspondence: Dr. A. Gopala Krishna, Banjara Cottage, 8-3-676/1/A/5/2 Yellareddyguda,
Hyderabad - 500073, 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
- Chandan K, Madnani N, Desai D, Deshpande R. AIDS - associated
Kaposi's sarcoma in a heterosexual male - A case report. Dermatol Online
J 8:19.
- Oxford text book of Oncology. In: Peckham M, Pinedo H,
Veronesi U, editors. 1995;1:900.
- Krown SE. MD, Memorial Sloan-Kettering Cancer Center Clinical
Characteristics of Kaposi's Sarcoma HIV In Site Knowledge Base Chapter
Published February 1997.
- Potouridou I, Katasambas A, Pantazi V, Armaneka M, Stavianeas
N, Stratigos J. Classic Kaposi's sarcoma in two young heterosexual men.
J Eur Acad Dermatol Venereol 1998;10:48-52.
- Susan E, Krown MD. AIDS - associated Kaposi's sarcoma,
biology and mana.gement. Med Clin N Am 1992;81:471.
© 2004 Indian Journal of Surgery.
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