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Our Dermatol Online. 2013; 4(1e): e1
Date of submission: 25.05.2013 / acceptance: 21.06.2013
Conflicts of interest: None
 

Case of classical sarcoma Kaposi in a non-HIV elderly Kashmiri male

Mohammad Hussain Mir1, Parvaiz Anwar Rather2, Farhana Bagdadi1, Sumaira Khursheed1,

Burhan Wani1

1Postgraduate Scholar Medicine, Sheri Kashmir Institute of Medical Sciences, Srinagar, Kashmir, India
2Department of Dermatology, STD & Leprosy, Govt. Medical College, SMHS Hospital, Srinagar, J&K, India
 

 

Abstract
Kaposi`s sarcoma (KS) is a tumor of endothelial origin, running a protracted clinical course. Four main types of KS have been described namely classical, endemic African, iatrogenic, human immunodeficiency virus (HIV) associated. There has been a spurt in the cases of KS after the HIV epidemic. We report a case of Kaposi sarcoma in a non-HIV elderly Kashmiri male and review the literature.
 
Key words:  Kaposi sarcoma; endothelial; non-HIV

 

Introduction
Kaposi sarcoma (KS) is a rare neoplasm of lymphatic endothelial cell origin [1], with multifocal cutaneous and extra-cutaneous involvement, predominantly affecting older individuals and running a protracted clinical course. Four major groups have been described namely classical, endemic African, iatrogenic, human immunodeficiency virus (HIV) associated [2]. There has been a rise in the cases of KS because of the HIV epidemic but non-HIV KS is rarer. We report KS in a non-HIV elderly Kashmiri male and review the literature.
 
Case Report
60 year old normo-tensive, non-diabetic male with no significant past history presented with 9 months duration of voilaceous eruptions over both feet. The eruptions started from a small area over dorsum of left foot and gradually progressed to whole of dorsal and plantar aspect, and lesions appeared on right foot also, over a period of 2-3 months. Eruptions were non-pruritic, painless, associated with swelling of feet. There were no lesions on rest of the body or ulceration/discharge from these lesions. There were no systemic features. There was nothing significant in the past, family or drug history. On examination, the patient was hemodynamically stable. There was left side inguinal lymphadenopathy measuring 3×2 cm, mobile, non tender, firm in consistency. Systemic examination was normal. Dermatological examination showed bilateral involvement of feet with multiple violaceous plaques, over dorsal and plantar aspect and also inter-digital clefts. Lesions were discrete, scaly, thick, non-blanching, non-tender, associated with edema of both feet (Fig. 1). Nails showed subungal hyperkeratosis, dystrophy. Hair and mucus membrane examination was normal. KOH examination and fungal culture from nails and skin was negative. With a possibility of lichenoid eruption, patient was put on topical steroids, with little improvement. The lesions were then subjected to biopsy with a possibility of Kaposi sarcoma. The histopathological examination under hematoxylin and eosin showed hyper-keratotic epidermis, with extensive lesion in dermis composed of capillaries, at places well formed and at places slit-like spaces. These spaces were infiltrated by chronic inflammatory infiltrate, favoring Kaposi Sarcoma (Fig. 2). Review of histopathology with immune histochemistry revealed Kaposi sarcoma with vascular channels highlighted by CD 31, CD 34 and showed nuclear staining with HHV8. The patient was referred to Medical Oncology department. Dissection and biopsy of lymph node showed partial involvement by Kaposi sarcoma with CD 20 reactive B cells. CECT chest and abdomen was normal. Hepatitis B, C and retroviral serology was non-reactive with HIV viral load < 20 copies/ ml. Absolute CD4+ count was 1072 (normal range 354- 1100) and Beta-2 micro-globulin was 2.58 mg/L, both normal. The patient was put on treatment with liposomal Doxorubicin (20mg/m2 IV infusion), repeated every 3 weeks. The patient has received total of 6 cycles so far and was on regular follow up. Significant improvement in the skin lesions has occurred (Fig. 3).
 
Figure 1. Bilateral involvement of feet with dry, discrete, voilaceous, scaly plaques, along with nail involvement
Figure 2. Photomicrograph showing hyperkeratotic epidermis, capillaries as well formed & slit like spaces in dermis, with chronic inflammatory infiltrate [H & E; 10X (A) & 40X (B)]. 
Figure 3. Resolving lesions after treatment with Doxorubicin. 
Discussion
Kaposi's sarcoma (KS), first described in 1872 by Hungarian dermatologist Moritz Kaposi [3], is a spindle cell, multifocal vascular tumor of endothelial origin [1]. Four groups are at risk to develop KS: elderly males of Mediterranean and Eastern European lineage; children and adults from central Africa; persons who have iatrogenic immune compromise; and homosexual men infected with human immunodeficiency virus (HIV) [4]. This has lead to the following groups of Kaposi sarcoma: classical KS, endemic African KS, iatrogenic KS and AIDS-KS2, with differences in clinical presentation and prognosis among the groups. Classic KS occurs in elderly men and is indolent. Endemic KS occurs in Sub-Saharan Africa. Epidemic KS refers to KS in HIV-infected individuals. It is aggressive and involves the skin, gastrointestinal and respiratory tracts. Iatrogenic KS occurs in individuals who are immune-suppressed because of organ transplantation and tends to be aggressive [5]. The clinical course of classic KS is benign and prolonged. In contrast to AIDS-associated KS, KS in non-HIV patients appears less aggressive, mostly limited to the skin and well-responsive to local or systemic therapeutic strategies. To date it remains unclear whether KS itself is a true malignancy or rather just a reactive proliferation [6]. The exact etio-pathogenesis remains unclear. The development of KS is a multistep, multi-factorial process involving genetic factors like origin, age, sex and acquired factors like infections, immune status; leading to production of various angiogenetic and inflammatory cytokines [7]. The main viral infections incriminated include CMV, HBV, HAV, HHV-6, HIV-tat, HPV and recently HHV-8 [8,9]. Most of the cases occur in the fifth and sixth decades of life. Kaposi's sarcoma usually starts as erythematous violaceous macules on the distal portions of lower extremities and lesions progress slowly to form plaques, nodules, fungi-form (verrucous) tumors or may erode and ulcerate. Visceral and/or mucosal involvement occurs in about 10% of these patients [10]. Histologically there is dermal proliferation of interlacing bundles of spindle cells and slit-like vessels with angulated vascular spaces. The spindle cell show variable nuclear pleomorphism and mitotic figures [11]. Treatment modalities depends on whether it is localized or widespread disease and include surgery, radiotherapy, chemotherapy such as vinca alkaloids, liposomal doxorubicin, danaurubicin, paclitaxel, immune therapy by interferon, interleukin-2, chorionic gonadotrophin, 9 cis-retinoic acid or imiquimod [12-15]. The elderly non-HIV Kashmiri male presented with typical clinical features of KS with relevant histo-pathological examination. The lesions resolved with Doxorubicin. We report this case to add to the list of non-HIV KS.
 
 
REFERENCES

1.Nadji M, Morales AR, Ziegles WJ, Penneys NS: Kaposi`s sarcoma: immunohistologic evidence for an endothelial origin. Arch Pathol Lab Med. 1981;105: 274-5.
2. Rappersberger K, Wolf K, Stingl G: Kaposi`s sarcoma. In: Fitzpatrick TB, Eisen AZ, Wolf K, Freedberg IM, Austen KF, editors. Dermatology in general medicine. 4th ed. New York: McGraw-Hill; 1993. p. 1244-56.
3. Kaposi M: Idiopathisches multiples Pigment sarkom der Haut. Arch Dermatol Syph. 1872;4:265-72.
4. Antman K, Chang Y: Kaposi's sarcoma. N Engl J Med. 2000;342:1027-38.
5. Montagnino G, Bencini PL, Tarantino A, Caputo R, Ponticelli C: Clinical features and course of Kaposi's sarcoma in kidney transplant patients: report of 13 cases. Am J Nephrol. 1994;14:121-26.
6. Goh SG, Calonje E: Cutaneous vascular tumours: an update. Histopathology. 2008;52:661-73.
7. Ensoli B, Sgadari C, Barillari G, Sirianni MC, Stürzl M, Monini P: Biology of Kaposi's sarcoma. Eur J Cancer. 2001;37:1251-69.
8. Senturk N, Sahin S, Ercis S, Kocagoz T, Atakan N: Human herpesvirus 8 (HHV-8) in non-HIV associated forms of Kaposi’s sarcoma from Turkey. Turk J Med Sci. 2001;31:503-08.
9. Chang Y, Caserman E, Pessin MS, Lee F, Culpepper J, Knowles DM, et al: Detection of herpes-virus like DNA sequences in AIDS-associated Kaposi`s sarcoma. Science. 1994;266:1865-9.
10. Iscovich J, Boffetta P, Franceschi S, Azizi E, Sarid R: Classic kaposi sarcoma: epidemiology and risk factors. Cancer. 2000;88:500-17.
11. Hong A, Lee CS: Kaposi's sarcoma: Clinico pathological analysis of HIV and non HIV associated cases. Pathol Oncol Res. 2002;8:31-5.
12. Toschi E, Sgadari C, Monini P, Barillari G, Bacigalupo I, Palladino C, et al: Treatment of Kaposi’s sarcoma-an update. Anticancer Drugs. 2002;13:977-87.
13. Di Lorenzo G, Kreuter A, Di Trolio R, Guarini A, Romano C, Montesarchio V, et al: Activity and safety of pegylated liposomal doxorubicin as first-line therapy in the treatment of non-visceral classic Kaposi's sarcoma: a multicenter study. J Invest Dermatol. 2008;128:1578-80.
14. Jan RA, Koul PA, Ahmed M, Shah S, Mufti SA, War FA: Kaposi Sarcoma in a Non HIV Patient. Int J Health Sci. 2008;2:153-56.
15. Jakob L, Metzler G, Chen KM, Garbe C: Non-AIDS Associated Kaposi's Sarcoma: Clinical Features and Treatment Outcome. PLoS One. 2011;6:e18397.

 



C O M M E N T S

 

Prof. Sundaramoorthy Srinivasan, Dr. Riswana Jasmine and Dr. Preethi K. – Chennai, India
This is to appreciate the wonderful clinical acumen in detecting Kaposi`s sarcoma in Non HIV individuals. This has given us an insight of the varied clinical presentation and treatment modalities that are available. However Histopathological photographs could have been more precise as we are unable to locate the capillary channels.
 
Dr. Harjeet Singh – Qatar
This is a nice presentation of Kaposi Sarcoma in a simple language giving the clear cut concept of every aspect of the KS. This case most likely may be due to iatrogenic immune compromise of the 60 years old male which predisposed him to develop this. Advancements in the development of immunotherapies can prevent the occurrence of these type of cases & also prevent the reoccurrence of the same disease in previously suffered person.
 
Prof. Hubert Daisley – Trinidad and Tobago
A section showing the classical histopathology of Kaposi sarcoma viz the proliferating capillaries and spindle cells, with the extravasation of red blood cells and the presence of old hemorrhage would have greatly enhanced this article.
 
 



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