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.

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.

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.

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.

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.

Anaplastic Kaposi's Sarcoma of the Adrenal in an HIV-negative Patient With Literature Review.

Kaposi's sarcoma (KS) is a peculiar tumor of viral etiology, with the HHV8 rhadinovirus playing a fundamental role in its development. Several epidemiological categories of KS have been identified, of which the sporadic, endemic, iatrogenic, and the epidemic are the main ones. Several histologic disease morphologies have been described, such as inflammatory, angiomatous, spindle cell, mixed, and the anaplastic (sarcomatous) subtypes. The skin of the limbs is most commonly affected, but any other organ or site may be involved. Microscopically KS may enter the differential diagnosis with several different entities, and for this purpose the immunohistochemical detection of the viral latent nuclear antigen-1 (LNA-1) may be crucial. Sporadic KS is usually benign, but rarely it may be aggressive. Anaplastic histology heralds an ominous course in any clinical context. We report a case of anaplastic retroperitoneal KS, occurring in an HIV-negative adult man. This patient presented with a huge left suprarenal mass, which was totally resected, and initially diagnosed as inflammatory leiomyosarcoma, because of the monomorphic spindle cell tumor morphology. After 12 years the tumor recurred locally as an unresectable mass, which was biopsied and examined. At the time of recurrence, the histologic slides of the primary tumor were reviewed, and the previous diagnosis was changed to that of atypical KS. Histologically the recurrent tumor showed both spindle cell and epithelioid appearances. Strongly diffuse HHV8/LAN-1 immunopositivity was documented in both tumors. The final diagnosis for the entire case was anaplastic KS. Then, the patient died in a few months.

Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis.

CASE REPORT: A 4-year-old boy with kaposiform lymphangiomatosis (KLA) developed progressive headaches and papilloedema and was diagnosed with pseudotumor cerebri initially treated with acetazolamide. Clinical deterioration prompted placement of a ventriculoperitoneal shunt. After the surgery, the child's condition has markedly improved. DISCUSSION AND CONCLUSIONS: A network of intracranial lymphatics is presently being investigated. Neuroimaging excluded KLA infiltration of the skull and/or meninges, leaving as the most plausible explanation for the child's pseudotumor cerebri the existence of an increase in intracranial venous pressure by venous compression at the thorax. To our knowledge, our case constitutes the first report of pseudotumor cerebri occurring in the context of KLA.

Outcome of second kidney transplantation in patients with previous post-transplantation Kaposi's sarcoma: A French retrospective study.

This retrospective study concerned 8 patients with post-transplantation Kaposi's sarcoma (pt-KS) after a first kidney transplant who later had a second kidney transplantation. Pt-KS was widespread, with lymph node or visceral involvement in 7 cases. Complete remission was observed in 6 cases and partial remission in 2. After the second kidney transplantation, only 2 cases showed recurrence of skin KS, one with previous complete remission of KS and one with partial remission. The mean delay between stability or complete remission of KS and retransplantation was 2.0 and 7.3 years in patients with and without relapse, respectively. Both recurrent cases showed complete KS remission after tapering immunosuppression therapy and/or switching a calcineurin inhibitor to a mammalian target of rapamycin inhibitor. We compared these 8 cases to 24 controls who had undergone 2 kidney transplantations but did not have KS, matching on sex, age and phototype. Cases and controls did not differ in graft function or survival. A second kidney transplantation may be possible after pt-KS and has acceptable risk, especially after a long complete remission of pt-KS.

Successful Treatment of Classic Kaposi Sarcoma With Long-Pulse Neodymium-Doped Yttrium Aluminum Garnet Laser: A Preliminary Study.

BACKGROUND: Kaposi sarcoma (KS) is a systemic disease that can present with cutaneous lesions with or without internal involvement, mostly caused by infection with human herpesvirus-8. The treatment options include surgical excision, cryotherapy, radiotherapy, intralesional chemotherapy, laser, and elastic stockings for the prevention of lymphedema. OBJECTIVE: This article presents 7 cases with classic KS treated with the long-pulse neodymium:yttrium-aluminum-garnet (Nd:YAG) laser. METHODS: Forty-nine lesions of 7 KS patients (5 stage 1, 2 stage 2A) were treated with Nd:YAG laser with a spot size of 4 to 6 mm and a fluence of 180 J/cm increased by 10 J/cm in the neighboring area to a maximum of 260 J/cm. The pulse sequencing was 1.5, and delay time was 5 milliseconds. Sessions were continued at 4-week intervals for 2 to 4 sessions. RESULTS: All patients exhibited clinical and histological improvement. One session was sufficient for small lesions, whereas coalescing and multicentric lesions required up to 4 sessions. All the lesions healed in 2 to 4 weeks, with the only complication being mild atrophic scars. CONCLUSION: With the advantage of penetrating into deeper sites than other lasers, long-pulse Nd:YAG is an efficient and safe local treatment alternative especially for papulonodular and deeper lesions located on bony structures.

Kaposi's sarcoma: An unusual penile lesion in a HIV negative patient.

Kaposi's sarcoma (KS) of the penis is a very rare lesion and it is usually observed in HIV-infected patients. We introduce a case of KS of the penis in a 75 years old HIV negative patient with a peripheral T-cell lymphoma. He came to our attention with a painful ulcerated red lesion on the glans that stretched from the urethral meatus to the coronal skin. This lesion was found to be a KS balanopreputial in the classical variant. Penile KS must be included in the differential diagnosis of genital diseases especially when the clinical features of the lesion are aspecific and diagnosis can be made histologically by performing a biopsy.

Clinical and pathological features and treatment of AIDS-related cutaneous Kaposi's sarcoma in Chinese Han patients.

This retrospective study aimed to observe the clinicopathological features and immunological phenotypes, and explore effective treatment and prognosis for 12 Chinese Han patients with acquired immunodeficiency syndrome-related cutaneous Kaposi's sarcoma. All 12 patients were human immunodeficiency virus-positive, and underwent the standard highly active antiretroviral therapy (HAART). Skin lesions mainly presented as purple, or rufous papules, or plaques; skin biopsy showed diffuse or flaky infiltration of spindle cells, active proliferation of slit-like vasculature, erythrocyte exudation, hemosiderin deposition, and inflammatory cell infiltration. Immunohistochemical analysis showed the expression of Ubiquitin C-terminal hydrolase L1 (+), and CD31 (+) in T-cells; factor VIII (+) and HHF-35 (+) in the proliferating vascular endothelial cells; vimentin (+) and S-100 protein (-) in the vessel wall; and CD34 (+++) in the spindle cells of 6 cases, with 1 case of negative CD34 expression. Four patients with confined lesions underwent surgery and microwave therapy, and received a favorable prognosis. Two patients with limited lesions underwent microwave therapy, and the lesions subsided. Of six patients with widely distributed sarcomas, five underwent microwave therapy and one received combined chemotherapy; five attained significant efficacy, and one died. There were no significant differences in the clinicopathological features and immunological phenotypes between the Chinese Han patients and those from other populations. Along with basal HAART, patients in early stages, with sarcomas <2 cm in diameter should undergo surgery and microwave therapy, while patients with sarcomas >2 cm in diameter should undergo chemotherapy and microwave therapy.

Suspected metastatic adrenocortical carcinoma revealing as pulmonary Kaposi sarcoma in adrenal Cushing's syndrome.

BACKGROUND: Kaposi sarcoma (KS) is a malignant disease most commonly diagnosed in the setting of a human immunodeficiency virus (HIV) infection and in patients receiving immunosuppressive treatment. Pulmonary KS has never been reported in association with endogenous Cushing's syndrome (CS). CASE PRESENTATION: A 60-year-old woman presented with symptoms and signs of CS. Adrenal CS was confirmed by standard biochemical evaluation. Imaging revealed a right adrenal lesion (diameter 3.5 cm) and multiple pulmonary nodules, suggesting a cortisol-secreting adrenal carcinoma with pulmonary metastases. The patient underwent right adrenalectomy with a pathohistological diagnosis of an adrenal adenoma. Subsequent thoracoscopic wedge resection of one lung lesion revealed pulmonary KS with positive immunostaining for human herpes virus 8 (HHV-8). HIV-serology was negative. Hydrocortisone replacement was initiated for secondary adrenal insufficiency after surgery. Post-operative follow up imaging showed complete remission of all KS-related pulmonary nodules solely after resolution of hypercortisolism. CONCLUSION: KS may occur in the setting of endogenous CS and may go into remission after cure of hypercortisolism without further specific treatment.

Kaposiform hemangioendothelioma complicated by Kasabach-Merritt phenomenon: ultrastructural observation and immunohistochemistry staining reveal the trapping of blood components.

Kaposiform hemangioendothelioma (KHE), a borderline tumor of endothelial origin, is associated with Kasabach-Merritt phenomenon, characterized by profound thrombocytopenia and consumptive coagulopathy resulting from the localized intravascular coagulation (LIC) in the tumor. Previous studies have suggested that the trapping of blood components, including platelets, may underlie the LIC in KHE. However, more evidence is needed to support this hypothesis. In this study, one case of a Chinese infant with a KHE in the left arm was complicated by Kasabach-Merritt phenomenon. The tumor was partially resected and the sample was used for ultrastructural observation and immunohistochemistry staining of Glut-1. Ultrastructural observation found the trapping of erythrocytes, platelets, macrophages, and lymphocytes in the slit-like channels of the tumor nodules, and phagocytic vesicles in the cytoplasm of neoplastic cells. Immunohistochemistry staining further showed numerous Glut-1(+) erythrocytes in the channels. In conclusion, our results provided compelling morphological evidence of the trapping of blood components in KHE, which may interpret the LIC in the tumor and subsequent consumptive coagulopathy.

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.