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Year : 2014  |  Volume : 10  |  Issue : 1  |  Page : 213-214

Curious Case of a Primary Intranodal Kaposi Sarcoma, Confirmed by Immunohistochemistry, in an HIV-positive African Male

1 Department of Pathology, Tata Memorial Hospital, Parel, Mumbai, Maharashtra, India
2 Department of Surgery, Prince Aly Khan Hospital, Mazagaon, Mumbai, Maharashtra, India
3 Department of Pathology, Prince Aly Khan Hospital, Mazagaon, Mumbai, Maharashtra, India

Date of Web Publication23-Apr-2014

Correspondence Address:
Bharat Rekhi
Department of Pathology, Tata Memorial Hospital, Dr. Ernest Borges Road, Parel, Mumbai - 400 012, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.131438

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How to cite this article:
Rekhi B, Rangwalla H, Chinoy RF. Curious Case of a Primary Intranodal Kaposi Sarcoma, Confirmed by Immunohistochemistry, in an HIV-positive African Male. J Can Res Ther 2014;10:213-4

How to cite this URL:
Rekhi B, Rangwalla H, Chinoy RF. Curious Case of a Primary Intranodal Kaposi Sarcoma, Confirmed by Immunohistochemistry, in an HIV-positive African Male. J Can Res Ther [serial online] 2014 [cited 2021 Oct 16];10:213-4. Available from: https://www.cancerjournal.net/text.asp?2014/10/1/213/131438


Kaposi sarcoma (KS), first described by Kaposi, [1] is currently defined as a locally aggressive, endothelial tumor or a tumor-like lesion that usually presents with cutaneous lesions, in form of multiple patches, plaques or nodules. It may affect mucosal sites but uncommonly affects lymph nodes and visceral organs. [2] It has characteristic histopathological features and is objectively confirmed by biopsy with positive immunohistochemical (IHC) expression of various endothelial markers and invariable nuclear expression of human herpes virus 8 (HHV8), latent nuclear antigen 1(LANA1). [2],[3] Primary intranodal KS is rare. Its rarity is further compounded as a result of a lower index of suspicion for this tumor presentation, especially when it presents as an isolate nodal mass with limited clinical details, in a referral case that creates a diagnostic challenge. Although cases of primary intranodal KS have been documented, only one of those was confirmed with IHC expression of HHV8. [4],[5],[6],[7],[8] Herein, we present this uncommon tumor presentation, objectively confirmed with IHC staining.

A 45-year-old, married Nigerian gentleman referred to us with complaints of fever of more than a month duration and bilateral inguinal lymphadenopathy. He was a human immunodeficiency virus (HIV)-seropositive patient. He did not have history of any skin lesions. Initially, he was treated with antibiotics, in view of possibility of an underlying bacterial infection. However, in view of significant lymphadenopathy, the subsequent clinical diagnosis considered was of a non-Hodgkin's lymphoma. He underwent excision biopsy of the nodes. There were no other accessible laboratory findings.

Conventional hematoxylin and eosin (H and E) stained sections form the lymph nodes showed near total replacement of nodal structure by nodules of proliferating spindly cells lining irregular, thin-walled vessels, as well as arranged in intersecting fascicles. At places, ectatic vessels were noted, along with extravasated red blood cells and focal hyaline droplets. The spindle cells exhibited moderate degree of nuclear atypia with several mitotic figures. Interspersed were plasma cells [Figure 1] a and b. Immunohistochemically, the tumor cells were diffusely positive for CD31 and HHV8 and were focally, weakly positive for CD34 [Figure 1]c and d. Diagnosis of intranodal KS was formed.
Figure 1: Histopathological findings (a) Cellular multinodular tumor replacing nodal structure with peripheral rim of lymphoid follicles (arrow heads). H and E, ×100, (b) Higher magnification displaying cellular spindle cell tumor with vascular channels and interspersed extravasated red blood corpuscles. H and E, × 400, (c) Tumor cells displaying diffuse CD31 immunostaining. Positively stained endothelial cells of thin blood vessels acting as internal controls (arrow heads) (DAB) ×400, (d) Tumor cells displaying intranuclear human herpes virus 8 immunostaining. 3'-3'diaminobenzidine tetrahydrochloride

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KS uncommonly involves the lymph nodes. [4],[5] However, it is one of the most common types of an angiosarcoma that metastasizes to the lymph nodes. This presentation is usually seen in young, homosexual men, in contrast to the present case who was a heterosexual, married male. [9] Moreover, such cases have associated skin lesions that were absent in our case, who presented with isolate febrile lymphadenopathy and was clinically suspected with non-Hodgkin's lymphoma. [6] Distinct histopathological features were helpful in developing an index of suspicion for diagnosis of a KS that was further solidified with diffuse expression of vascular markers like CD31 and CD34. Some of these histopathological features and immunoprofile can be identified in a Kaposiform hemangioendothelioma (KHE). However, that tumor invariably occurs in children; may or may not be associated with Kasabach-Merritt syndrome and lacks significant nuclear atypia and/or mitotic figures, on biopsy. [10] Histopathologically, a KS and a KHE can be objectively differentiated by application of HHV8 LANA1 marker, nuclear expression of which is present in the former and lacking in the latter. Aforementioned histopathological features and immunoprofile ruled out a spindle cell inflammatory pseudotumor that is composed of spindle cells without significant atypia and absent mitotic figures. Diffuse CD31 and HHV8 immunoreactivity also militated against diagnosis of bacillary angiomatosis that formed as another differential diagnosis. Rarely, intranodal KS has also been found to be coexistent with mycobacterial infection and Hodgkin lymphoma in district case reports. [6],[7],[8],[11] Of these, only a single case that occurred in another African male, who additionally had skin lesions, documented by Lanjewar et al., [8] was confirmed with positive HHV8 immunostaining. Apart from an intranodal KS, no additional histopathological findings were seen in the present case. This patient was a candidate for highly active antiretroviral therapy treatment. However, he left for his home country for further management.

To sum up, the present case is the second documentation of a primary intranodal KS from our country, but occurring in an African male , objectively confirmed on biopsy with positive HHV8 LANA1 immunostaining. Presence of vascular pattern and spindly cells, especially in the lymph node of an HIV-positive patient are useful diagnostic ''clues'' for this diagnosis that should be reinforced with necessary IHC markers, including HHV8. Unfortunately, our patient was lost to follow-up.

 > References Top

1.Braun M. Classics in Oncology. Idiopathic multiple pigmented sarcoma of the skin by Kaposi. CA Cancer J Clin 1982;32:340 - 7.  Back to cited text no. 1
2.Mentzel T, Knuutila S, Lamovec J. Kaposi sarcoma. In: Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F, editors. World Health Organization (WHO) Classification of Tumors of Soft Tissue and Bone. Lyon: IARC Press; 2013. p. 151-3.  Back to cited text no. 2
3.Patel RM, Goldblum JR, Hsi ED. Immunohistochemical detection of human herpes virus-8 latent nuclear antigen-1 is useful for the diagnosis of Kaposi sarcoma. Mod Pathol 2004;17:456-60.  Back to cited text no. 3
4.Schwartz RA, Brenden LD, Breeden JH, Lambert WC. Indolent lymphadenopathic Kaposi's sarcoma. J Surg Oncol 1987;34:243-7.  Back to cited text no. 4
5.Ioachim HL, Adsay V, Giancotti FR, Dorsett B, Melamed J. Kaposi›s sarcoma of internal organs. A multiparameter study of 86 cases. Cancer 1995;75:1376-85.  Back to cited text no. 5
6.Croxson TS, Ebanks D, Mildvan D. Atypical mycobacteria and Kaposi's sarcoma in the same biopsy specimens. N Engl J Med 1983 16;308:1476.  Back to cited text no. 6
7.Bodhireddy H, Rivas S, Seshadri T. Coexistent Kaposi›s sarcoma and atypical mycobacterial infection involving lymph node: A case report and review of literature. Indian J Pathol Microbiol 2010;53:805-7.  Back to cited text no. 7
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8.Lanjewar DN, Lanjewar SD, Chavan G. Coexistent lymphoma with tuberculosis and Kaposi›s sarcoma with tuberculosis occurring in lymph node in patients with AIDS: A report of two cases. Indian J Pathol Microbiol 2010;53:551-4.  Back to cited text no. 8
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9.Finkbeiner WE, Egbert BM, Groundwater JR, Sagebiel RW. Kaposi›s sarcoma in young homosexual men: A histopathologic study with particular reference to lymph node involvement. Arch Pathol Lab Med 1982;106:261-4.  Back to cited text no. 9
10.Zukerberg LR, Nickoloff BJ, Weiss SW. Kaposiform hemangioendothelioma of infancy and childhood: An aggressive neoplasm associated with Kasabach-Merrittt syndrome and lymphangiomatosis. Am J Surg Pathol 1993;17:321-8.  Back to cited text no. 10
11.Carbone A, Volpe R. Kaposi›s sarcoma in lymph nodes concurrent with Hodgkin›s disease. Am J Clin Pathol 1983;80:228-30.  Back to cited text no. 11


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