HIV with initial ocular Kaposi sarcoma / HIV com sarcoma de Kaposi ocular inicial

ABSTRACT We present an interesting case of a 62-year-old black female, presented to the ophthalmological hospital with a little "nevus" on the left eye previously visualized at the mirror, with one month of development. Physical examination with slit lamp (biomicroscopy) showed a group of painless veins, with vascular redness, and a mass nodular aspect in the mid temporal bulbar conjunctiva, of approximately 2mmx4mm.

RESUMO Apresentamos o interessante caso de uma mulher negra de 62 anos, que deu entrada no hospital oftalmológico com um pequeno nevo no olho esquerdo previamente visualizado ao espelho, com 1 mês de evolução. O exame físico com lâmpada de fenda (biomicroscopia) mostrou um grupo de veias indolor e vermelhidão vascular, com uma massa de aspecto nodular na conjuntiva bulbar temporal média, de aproximadamente 2mmx4mm.

Rare adult Kaposiform hemangioendothelioma with multiple-bone invasion - clinical experience and literature review.

BACKGROUND: Kaposiform hemangioendothelioma (KHE) is a borderline vascular tumor between hemangioma and malignant angiosarcoma. While KHE has strong local invasion with rare spontaneous regression, it is not observed with distant metastasis. Even if KHE is asymptomatic or without the Kasabach-Merritt phenomenon (KMP), bone or joint invasion should clearly receive proactive treatment. KHE commonly affects infants/children but is rarely seen in adults. CASE REPORT: We reported a rare adult KHE case with an invasion of >10 separate forearm/hand bones, who underwent multiple-lesion resection and finger amputation after tumor recurrence. Tumor recurrence and KMP were not observed during the 6-month follow-up after the final operation. During the hospitalization and follow-up period, the patient only received medications for infection prevention and pain relief. CONCLUSIONS: Multiple resectable lesions were found in the distal limb, for which complete resection might not present typical features (high-intensity T2-weighted MRI), which might fail to detect all KHE lesions. Therefore, complete excision is not optimal for multiple resectable KHE lesions.

Kaposiform hemangioendothelioma of the heart: a case report and literature review.

Kaposiform hemangioendothelioma is a rare tumour of vascular origin that rarely occurs in the heart. We provided a rare case of a 26-day-old infant with tachypnoea. Echocardiography showed a solid tumour in the pericardial cavity and a large amount of pericardial effusion. The solid tumour was confirmed by surgery, and the pathology was kaposiform hemangioendothelioma. We analysed this case and reviewed the related literature to explore the clinical features and echocardiographic manifestations to improve the understanding, diagnosis, and treatment of this disease for clinicians and sonographers.

A Case of a Rare Histologic Variant: Cavernous Hemangioma-Like Kaposi Sarcoma.

ABSTRACT: Kaposi sarcoma (KS) is an endothelial tumor associated with human herpesvirus 8. Cutaneous lesions can present with pink or purple patches, plaques, and nodules which can be ulcerated. The main subtypes of KS generally have similar histologic appearances, with spindle cells and expression of human herpesvirus 8 being characteristic features. However, various histologic variants have been reported. We present the case of a 55-year-old man with cutaneous KS with cavernous hemangioma-like histological features. Cavernous hemangioma-like KS is a rare morphologic type of KS, with only a handful of cases reported in the literature.

Pleural Kaposi sarcoma: an unusual clinical case.

Kaposi sarcoma is a low-grade mesenchymal tumor associated with human herpesvirus-8. Here we describe the case of a 37-year old woman, who underwent to kidney and liver transplant for congenital hepatic fibrosis and bilateral polycystic kidney, with successive immunosuppressive therapy. After 5 years from first transplant, she developed cutaneous, mucosal, pleural and nodal localizations of Kaposi sarcoma, without lung lesions. Because of an initial clinical presentation with an important nodal and pleural involvement, a diagnosis of a lymphoproliferative disease was suspected. Pathological examination of the pleural sample allowed to exclude lymphoproliferative neoplasia and was consistent with Kaposi sarcoma. Subsequently involvement of other sites was diagnosed as expression of diffuse disease. The interest of this case lays in the unusual clinical presentation which can lead to diagnostic pitfalls when evaluating pleural biopsies.

Kaposi sarcoma of the adrenal gland: A report of two cases with a review of the literature.

Kaposi sarcoma (KS) is a low-grade vascular neoplasm commonly involving mucocutaneous sites, while adrenal gland involvement is exceptional. The anaplastic variant is a rare entity characterized by marked cellular pleomorphism, increased mitosis, worse prognosis, and an increased metastatic potential. The diagnosis remains on histology and immunohistochemistry. We describe two cases of primary adrenal KS to report on this exceptional presentation of KS: the first case is a 67-year-old female with anaplastic KS wit fatal outcome. The second case is a 56-year-old male who presented a classic KS of the adrenal gland. Until now, no standard efficient treatment regimens have been clearly identified. There is a need for a further understanding of anaplastic KS's biology, and collecting a sizable patient cohort remains essential to review treatment outcome.

Kaposiform Hemangioendothelioma of the Oral Cavity: A Rare Tumor with an Unusual Location.

Kaposiform hemangioendothelioma is a rare neoplasm with intermediate malignant behavior, mainly affecting infants and children. Involvement head and neck is uncommon, and there are only four cases reported in the oral cavity and oropharynx. Microscopically, it is characterized by a vascular proliferation permeated by spindle-to-ovoid cells resembling Kaposi sarcoma. Immunohistochemically, the tumor is positive for CD31, CD34 and negative for D2-40. Herein we present a rare case of intraoral Kaposiform hemangioendothelioma in a 10-year-old boy.

Kaposiform Hemangioendothelioma: clinicopathological characteristics of 8 cases of a rare vascular tumor and review of literature.

BACKGROUND: Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignant potential which shows locally aggressive growth but only rarely metastasizes. It is mostly considered to be a tumor of pediatric population but its occurrence in the adults is not uncommon as once considered. Histologically, KHE can mimic other soft tissue neoplasms of different behaviors (e.g. Kaposi Sarcoma, hemangioma) and establishing the correct diagnosis is important for appropriate treatment. Herein, we describe the clinicopathological features of 8 cases of KHE which will be helpful in making their diagnosis. METHODS: We reviewed pathology reports, microscopy glass slides and obtained follow up information about 8 cases of KHE which were diagnosed at our institution from January 2008 till June 2020. Immunohistochemical stain for HHV8 was also performed. RESULTS: Age ranged from 7 months to 25 years. Seven patients were less than 20 years of age and one patient was 25 years old. Equal gender distribution was observed. Extremities were the most common sites of involvement, followed by head and neck, pancreas and ischiorectal region. 2 cases were resection specimen and all others were incisional biopsies. The largest tumor size was 5.5 cm in one of the resections. The incisional/fragmented tissues were all less than 5 cm in aggregate. Most cases showed predominance of nodular growth and a minor component of spindle cell population along with lymphangiomatosis like vascular channels, with evidence of microthrombi in 2 cases. Few multinucleated giant cells were observed in 2 cases. None of the cases exhibited significant nuclear atypia or mitotic activity. One of the cases arising in dermis showed underlying bone involvement. HHV8 was negative in 7/7 cases. CONCLUSIONS: KHE can also involve adult population and it should always be considered in the differential diagnoses of a vascular lesion. Presence of multinucleated giant cells is a rare finding. Knowledge about histological features and potential mimics is helpful in avoiding misdiagnosis.

Kaposiform haemangioendothelioma of duodenum in a neonate.

Kaposiform haemangioendothelioma, an endothelial borderline tumour, is typically seen in childhood involving extremities. It has been closely associated with a consumptive coagulopathy state, Kasabach-Merritt phenomenon (KMP). Extracutaneous involvement is uncommon. Intestinal involvement is quite uncommon and can masquerade as an acute abdomen. A 24-day-old neonate presented with bilious vomiting and fever for 5 days. Sections from the resected gangrenous duodenum contained a submucosal tumour composed of infiltrating nodules of slit-like or crescentic CD34-positive spindled-to-flattened endothelial-lined vascular spaces along with zones of fibrosis. No nuclear pleomorphism or necrosis identified. The findings were classic example of kaposiform haemangioendothelioma with an absence of any deranged coagulation profile. The index case raises interest given its congenital incidental presentation at an uncommon site, like duodenum, and absence of coexistent KMP.

Rare Presentation of Kaposi's Sarcoma in an HIV-Negative Patient.

Kaposi's sarcoma (KS) in the United States is prevalent in Eastern European or Mediterranean males. KS in HIV-negative patients is becoming increasingly prevalent and should not be limited to the 4 subtypes: classic, endemic, iatrogenic immunosuppression, and epidemic. We present a rare pedal presentation of KS in an immunocompetent, HIV-negative man who has sex with men (MSM) and was followed for 12 months. The prevalence of KS in HIV-negative MSM is slowly rising and fits into the rare, fifth subtype.

Isolated limb perfusion is an effective treatment modality for locally advanced Kaposi sarcoma of the extremities.

INTRODUCTION: Kaposi sarcoma (KS) is a rare soft tissue sarcoma. In case of locally advanced disease, mutilating surgery such as amputations or major reconstructive procedures are sometimes inevitable. The aim of this study was to evaluate the effectiveness of isolated limb perfusion (ILP) in patients with locally advanced KS of the extremities. MATERIAL AND METHODS: All patients who underwent ILP for KS between 1996 and 2018 at Erasmus MC, Rotterdam were identified. Clinical data was obtained from either a prospectively maintained database or retrospective assessment of patient files. RESULTS: A total of 14 primary ILP's were performed in 11 patients. Median follow-up from primary ILP was 30 months (range, 5-98). The overall response rate of primary ILP was 100%, with a complete response (CR) rate of 50%. Only minimal local toxicity (Wieberdink I-III) was observed. Local progressive disease occurred after eight primary ILP's (57%) with a median local progression free survival (PFS) of 18 months (95% confidence interval [CI]: 7.0-28.9). Subsequently, four (46%) patients received a total of 5 recurrent ILP's. After the recurrent ILP on the same leg, the overall response rate was 75% and a CR-rate of 50%. One patient needed amputation post-operatively resulting in a limb salvage rate of 91%. One (9%) patient developed metastases four months after ILP. CONCLUSIONS: ILP is a highly effective treatment modality with very limited morbidity rates for patients with locally advanced KS of the extremity. ILP should be considered as a treatment modality for locally advanced KS of the extremities.

Human herpesvirus 8-associated colonic Kaposi's sarcoma during vedolizumab treatment in ulcerative colitis: a case report and review of the literature.

BACKGROUND: Kaposi's sarcoma (KS) is a rare vascular tumor associated with human herpesvirus (HHV)-8 infection. One of the variants of KS is defined iatrogenic and is overall reported in transplanted patient but also, although less frequently, in patients treated with long-standing immunosuppressive therapy, such as in inflammatory bowel disease including ulcerative colitis and Crohn's disease. CASE PRESENTATION: Herein, we report the first case of KS in a human immunodeficiency virus (HIV)-negative 47-year old male with UC after treatment with the α4-ß7 integrin inhibitor vedolizumab (VDZ). The patient underwent to colectomy for a medical refractory disease and the histological examination of the surgical specimen showed the typical findings of KS together with the HHV-8 positivity. The patient achieved a good health status, without any sign of disease recurrence. CONCLUSIONS: In the present case, we can assume that VDZ may have promoted the reactivation of a latent HHV-8 infection endowed with oncogenic potentialities and, in turn, the onset of KS. We also briefly reviewed all the cases of KS in HIV-negative patients with inflammatory bowel disease.

Upper arm life-saving amputation of a 12 day-old neonate due to extensive vascular tumor of the upper extremity.

Vascular tumors in neonates are mostly benign; however, locally aggressive voluminous forms may destabilize the hemodynamics of a neonate. Herein, we present an unusual case of a neonatal giant vascular tumor in the right upper extremity, causing a consumption coagulopathy and acute deterioration of vital signs. The patient required mechanical ventilation, inotropic support, and administration of blood products by the seventh day. Vascular embolization attempts failed to improve the general condition of the patient. Due to the deteriorating and life-threatening general condition of the patient, amputation around the upper arm level occurred under emergency conditions on the twelfth day. The patient's hemodynamic parameters were regained immediately, with neither inotropic agents nor blood products required after the second postoperative day. Clinical and pathological diagnosis revealed kaposiform hemangioendothelioma. Patient monitoring proceeded until the age of 15 months, with no local recurrence around the stump or soft tissue coverage complications. Therefore, since other treatment options failed, the early amputation decision was life-saving.