|Year : 2018 | Volume
| Issue : 3 | Page : 295-298
Kaposi's sarcoma of the colon presenting with diarrhea and weight loss
Rodrigo Duarte-Chavez1, Lauren E Stone2, Leah N Grandi2, Ayaz Matin1, Kimberly J Chaput1, Santo Longo3, Berhanu M Geme1
1 Department of Internal Medicine, St. Luke's University Health Network, Bethlehem, USA
2 Lewis Katz School of Medicine at Temple University, Philadelphia, Pennsylvania, USA
3 Department of Pathology, St. Luke's University Health Network, Bethlehem, USA
|Date of Submission||11-Feb-2018|
|Date of Acceptance||14-Apr-2018|
|Date of Web Publication||24-Dec-2018|
Dr. Berhanu M Geme
Department of Internal Medicine, St. Luke's University Hospital Network, 801 Ostrum Street, Bethlehem, Pennsylvania 18015
Source of Support: None, Conflict of Interest: None
Kaposi's sarcoma (KS) is a rare tumor caused by human herpesvirus-8. The lesions present as reddish to purple slow growing macules that become nodular, typically affecting the extremities and sometimes associated to mucosal, visceral or lymph node involvement. KS is very prevalent in immunosuppressed patients and Sub-Saharan Africa. Gastrointestinal (GI) involvement is more common in the upper GI tract and usually presents as bleeding. We present a case of a 60-year-old male presenting with Kaposi's sarcoma of the colon, with diarrhea and weight loss as the clinical manifestation of the disease.
The following core competencies are addressed in this article: Patient care, Medical knowledge.
Keywords: Colon, diarrhea, HIV, Kaposi's sarcoma, Human Herpesvirus-8
|How to cite this article:|
Duarte-Chavez R, Stone LE, Grandi LN, Matin A, Chaput KJ, Longo S, Geme BM. Kaposi's sarcoma of the colon presenting with diarrhea and weight loss. Int J Acad Med 2018;4:295-8
|How to cite this URL:|
Duarte-Chavez R, Stone LE, Grandi LN, Matin A, Chaput KJ, Longo S, Geme BM. Kaposi's sarcoma of the colon presenting with diarrhea and weight loss. Int J Acad Med [serial online] 2018 [cited 2019 Dec 12];4:295-8. Available from: http://www.ijam-web.org/text.asp?2018/4/3/295/248330
| Introduction|| |
Kaposi's sarcoma (KS) presents as reddish-purple slow-growing macules that become nodular. The lesions typically affect the skin of the extremities but can progress to nodal, mucosal, and visceral involvement and currently account for 0.1% of all malignancies., In 1872, Moritz Kaposi described five cases of a pigmented sarcoma of the skin with an aggressive presentation. In 1981, at the time of the AIDS epidemic, an aggressive form of KS with an increased frequency of visceral involvement was recognized. In 1994, human herpesvirus-8 (HHV-8) was identified in a skin lesion of a patient with KS. HHV-8 is transmitted predominantly by saliva and is the only known etiology of KS., The seroprevalence of HHV-8 in asymptomatic patients ranges from 1.3% in Asia to 50%–70% in Africa., In AIDS-related KS, the migration from localized disease to generalized spread does not necessarily occur, and the multifocal lesions are rather secondary to multicentricity. In general, gastrointestinal (GI) involvement has an incidence ranging from 40% to 66% and is the most common type of visceral involvement.,, The upper GI tract, especially the gastric submucosa, is the most common site involved., The tumor is usually silent, but as it grows, it can manifest as occult or massive hemorrhage, malabsorption, abdominal pain, bowel obstruction, or perforation. The colon is less frequently affected and is usually associated with extensive skin involvement and poor prognosis.,,,
| Case Report|| |
A 60-year-old male immigrant from Liberia, living in the United States for the last decades, presented to the office with inguinal pain and diarrhea of 1-week duration. He denied fever, bloody stools, recent sick contacts, recent travel, and use of intravenous drugs and was currently in a monogamous heterosexual relationship. Routine blood work was ordered and showed normal white blood cell count with monocytosis in the differential. The rest of the results were normal except for elevated total protein with hypoalbuminemia. Urine analysis was positive for protein. One week later, the patient persisted with diarrhea and adenopathy. Routine stool culture for enteropathogenic bacteria and Clostridium difficile toxin was ordered and was negative. Computed tomography was ordered to evaluate the adenopathy. One lymph nodes (LN) measuring 14 mm × 16 mm was found on the right groin and small mesenteric LNs in the abdomen. Serum protein electrophoresis (SPEP) and urine protein electrophoresis (UPEP) were also obtained. UPEP was normal and SPEP reported as nonspecific.
One month later, the patient presented with persistent diarrhea and seven-pound weight loss. On physical examination, LN on the groin was decreasing in size compared to the previous visits. In the setting of the previous findings and persistent diarrhea, an esophagogastroduodenoscopy (EGD) and colonoscopy were ordered. EGD revealed normal findings. During colonoscopy, a single polypoid lesion, 5 mm, was identified in the hepatic flexure and resected using cold biopsy polypectomy. The resected polyp was positive for KS [Figure 1] and [Figure 2]. Due to the known association between KS and AIDS, the patient was tested for HIV and was positive. CD4 in this patient was 176 cells/μL but initially refused antiretroviral treatment. The patient previously tested negative for HIV 13 years ago. On interval visit 2 weeks later after the polyp removal, the diarrhea had resolved. After extensive counseling, the patient agreed to start treatment for HIV infection.
|Figure 1: Beneath the colonic glands (arrowhead) bundles of tumor spindle cells invade the lamina propria and submucosa. Red blood cells have extravasated between the tumor cells (H and E, ×200)|
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|Figure 2: The pleomorphism of the tumor nuclei and the extracapillary extravasation of red blood cells are more evident at greater magnification (H and E, ×400)|
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| Discussion|| |
Endoscopically, the lesions of KS have been described as hemorrhagic patches and maculopapular, polypoid, umbilicated, nodular, and large exophytic masses [Table 1].,,,,, KS is a multicentric angioproliferative tumor and has the ability to regress or progress depending on the immune status of the host. Histology shows inflammation, angioma, or fibroma and characteristic spindle cells with vascular slits and hemorrhage or hemosiderin deposits., KS is considered a tumor of lymphatic endothelial cells, and based on immunolabeling, it expresses ETS-related gene, CD34, CD31, Fli-1, vascular endothelial growth factor receptor-3, and D2-40 as well as HHV-8 antibodies [Figure 3]., Currently, four subtypes of KS have been categorized.
|Figure 3: Immunohistochemical identification of the endothelial marker ETS-related gene in the spindled nuclei, as a black-brown stain. Confirmation of the human herpesvirus-8 also appears as a nuclear stain, identical to this picture|
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ClassicKS is more prevalent in people of Jewish or Mediterranean descent, with men more affected than women and usually at older age. The disease can present in the absence of immunosuppression but is usually associated with malignancy of lymphoreticular or hematopoietic origin. It typically presents as red-to-purple nodules in the extremities and progresses centripetally over years or decades, reaching visceral involvement in 10%–18% of cases.,
Endemic KS is very common in Sub-Saharan Africa, and this variant is very aggressive and affects younger patients. It is one of the most common cancers in children in Africa. Patients present with localized disease in the form of nodules or plaques and lymphedema.
Iatrogenic KS affects patients who are on immunosuppressive therapy for different conditions. People of Jewish and Mediterranean origin are also more commonly affected. Half of the cases will present in the first 3 years after initiating immunosuppression. This variant is more aggressive, presenting with visceral involvement even in the absence of skin involvement.,
Epidemic KS is more common in homosexual men with AIDS and has an aggressive course. It is usually associated to life-threatening opportunistic infections and extensive involvement of the skin, LN, and viscera. It affects more commonly the upper half of the body than the other variants. Before highly active antiretroviral therapy (HAART), KS was found in 25% of the patients with AIDS. The incidence has declined since HAART was introduced. However, it can still occur in patients with a normal CD4 count., It continues to be the most common HIV-related tumor in Africa, but non-AIDS-defining cancers have become more common in the developed world.
KS is not considered a curable tumor. In patients with single lesions, simple excision offers satisfactory treatment. Radiation and topical chemotherapy are useful when few lesions are present in a discrete area. For patients with systemic disease, options include surgery, chemotherapy, and radiotherapy, either as single treatment or as combined therapy. In patients with AIDS, the use of HAART slows progression of the sarcoma and in some cases causes regression of the tumor. Immunotherapy with interferon and antiangiogenic monoclonal antibodies has demonstrated fair response rates. Only ganciclovir has proven to be effective in suppressing HHV-8. In the subgroup of patients with iatrogenic KS, stopping the immunosuppressive therapy has been proven to improve or cure KS.,,
| Conclusions|| |
While cutaneous KS is very common in patients with AIDS, isolated colonic lesions are rare and they may precede the manifestation of skin lesions. If present, they are usually identified after an episode of bleeding. Isolated lesions presenting only with diarrhea have not been commonly described. The ability to identify the potential implications of vascular lesions in the GI tract is of utmost importance since early diagnosis of KS will improve the prognosis, particularly in patients with AIDS, where HAART halts KS progression or causes its regression.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent whereby the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patient(s) understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
Ethical conduct of research
IRB/Ethics approval is not required because it does not involve human or animal experimental designs.
| References|| |
Querido S, Sousa HS, Pereira TA, Birne R, Matias P, Jorge C, et al.
Gastrointestinal bleeding and diffuse skin thickening as Kaposi sarcoma clinical presentation. Case Rep Transplant 2015;2015:424508.
Antman K, Chang Y. Kaposi's sarcoma. N Engl J Med 2000;342:1027-38.
Byrne JJ. Kaposi's sarcoma. N Engl J Med 1962;266:337-8.
Svrcek M, Tiret E, Bennis M, Guyot P, Fléjou JF. KSHV/HHV8-associated intestinal Kaposi's sarcoma in patient with ulcerative colitis receiving immunosuppressive drugs: Report of a case. Dis Colon Rectum 2009;52:154-8.
Santana-Davila R, Bhatia S, Chow LQ. Harnessing the immune system as a therapeutic tool in virus-associated cancers. JAMA Oncol 2017;3:106-12.
Kahl P, Buettner R, Friedrichs N, Merkelbach-Bruse S, Wenzel J, Carl Heukamp L, et al.
Kaposi's sarcoma of the gastrointestinal tract: Report of two cases and review of the literature. Pathol Res Pract 2007;203:227-31.
La Ferla L, Pinzone MR, Nunnari G, Martellotta F, Lleshi A, Tirelli U, et al.
Kaposi' s sarcoma in HIV-positive patients: The state of art in the HAART-era. Eur Rev Med Pharmacol Sci 2013;17:2354-65.
Friedman SL, Wright TL, Altman DF. Gastrointestinal Kaposi's sarcoma in patients with acquired immunodeficiency syndrome. Endoscopic and autopsy findings. Gastroenterology 1985;89:102-8.
Saltz RK, Kurtz RC, Lightdale CJ, Myskowski P, Cunningham-Rundles S, Urmacher C, et al.
Kaposi's sarcoma. Gastrointestinal involvement correlation with skin findings and immunologic function. Dig Dis Sci 1984;29:817-23.
Rodriguez S, Zapatier J, Allende D, Schneider A. Kaposi's sarcoma: An unusual cause of gastric outlet obstruction. ACG Case Rep J 2013;1:19-21.
Richardson JD. Vascular lesions of the intestines. Am J Surg 1991;161:284-93.
Gordon FH, Watkinson A, Hodgson H. Vascular malformations of the gastrointestinal tract. Best Pract Res Clin Gastroenterol 2001;15:41-58.
Regula J, Wronska E, Pachlewski J. Vascular lesions of the gastrointestinal tract. Best Pract Res Clin Gastroenterol 2008;22:313-28.
Yano T, Yamamoto H. Vascular, polypoid, and other lesions of the small bowel. Best Pract Res Clin Gastroenterol 2009;23:61-74.
Olsen EG, Wellwood JM. Haemangiopericytoma of the small intestine. A report of three cases. Br J Surg 1970;57:66-9.
Voltaggio L, Montgomery E. Polypoid stromal lesions of the intestines. Histopathology 2015;66:88-101.
Yaskiv O, Rubin BP, He H, Falzarano S, Magi-Galluzzi C, Zhou M, et al.
ERG protein expression in human tumors detected with a rabbit monoclonal antibody. Am J Clin Pathol 2012;138:803-10.
Weissmann A, Linn S, Weltfriend S, Friedman-Birnbaum R. Epidemiological study of classic Kaposi's sarcoma: A retrospective review of 125 cases from Northern Israel. J Eur Acad Dermatol Venereol 2000;14:91-5.
Neumann H, Vieth M, Mönkemüller K. Infectious colon polyp in patient with human immunodeficiency virus. Endoscopy 2014;46 Suppl 1:E439-40.
Girelli CM, Serio G, Rocca E, Rocca F. Refractory ulcerative colitis and iatrogenic colorectal Kaposi's sarcoma. Dig Liver Dis 2009;41:170-4.
[Figure 1], [Figure 2], [Figure 3]