1/50. Intramuscular spindle cell hemangioendothelioma.Spindle cell hemangioendothelioma occurring in skeletal muscle is extremely rare. No reported studies have performed an imaging evaluation of intramuscular spindle cell hemangioendothelioma. We report on such a tumor arising in an unusual site, the right extensor digiti minimi, in a 46-year-old woman. An en bloc resection was performed and the patient has been disease free for 8 years. Radiologic imaging in the present case showed similar findings to those described in intramuscular hemangioma.- - - - - - - - - - ranking = 1keywords = spindle cell, spindle (Clic here for more details about this article) |
2/50. Spindle cell hemangioma.A 27-year-old woman presented with multiple nodules closely grouped on her right upper distal extremity. The lesions, dating from childhood, increased slowly in time. Microscopic examination of one nodule showed the histologic features of spindle cell hemangioendothelioma (SCH). At the periphery of the nodule there were also some features of the so-called sinusoidal hemangioma. Clinically, SCH can present as a solitary lesion or as multiple lesions in zonal distribution. When the lesions are multiple, the diagnosis of Maffucci's syndrome should be considered. SCH may be interpreted as a reactive process secondary to thrombosis and recanalization occurring in angiomatous lesions with different clinical presentations. Spindle cells are probably mesenchymal cells modified by blood pressure. For this entity the term hemangioma seems to be preferable to that of hemangioendothelioma.- - - - - - - - - - ranking = 0.2keywords = spindle cell, spindle (Clic here for more details about this article) |
3/50. Isolated oropharyngeal Kaposi's sarcoma in non AIDS patient: differences and similarities with spindle-cell haemangioendothelioma.Vascular tumours rarely affect the oropharynx and overall they can represent a diagnostic challenge since their clinico-histopathological patterns are not always clear. This case report, of an isolated pharyngeal vascular proliferation, allowed the authors to analyse the similarities and differences between Kaposi's sarcoma and spindle-cell haemangioendothelioma. Moreover, it emphasizes the importance of diagnostic tools, such as the human herpesvirus 8 (HHV8) marker, that sometimes may represent the only reliable test for clearly establishing the diagnosis.- - - - - - - - - - ranking = 0.23011990455154keywords = spindle (Clic here for more details about this article) |
4/50. Kaposiform hemangioendothelioma in adult. Report of a case with amianthoid-like fibrosis and angiectases.A rare case of kaposiform hemangioendothelioma in adult is reported. The 11 x 7 x 5 cm tumor was excised from deep subcutis of the abdominal region in 37-year-old man. No signs of kasabach-merritt syndrome or lymphangiomatosis were present. Besides typical pattern of kaposiform hemangioendothelioma, following unusual features were found: dilated vessels producing a gross impression of spindle cell hemangioma, areas of amianthoid-like fibrosis, and diffuse immunoreactivity for CD99. The differential diagnosis included mainly spindle cell hemangioma (hemangioendothelioma), hemangiopericytoma-like solitary fibrous tumor, and Kaposi's sarcoma.- - - - - - - - - - ranking = 0.4keywords = spindle cell, spindle (Clic here for more details about this article) |
5/50. Reactive angioendotheliomatosis secondary to dermal amyloid angiopathy.Reactive angioendotheliomatosis (RAE) is a rare benign cutaneous vascular proliferation characterized by intravascular hyperplasia of endothelial cells and tuft-like proliferation of vessels. A 75-year-old man had erythematous and violaceous macules, some stellate and others arranged in a livedoid pattern, evolving toward necrosis with central areas having an "atrophie blanche" appearance spread on the trunk, inguinal folds, and right thigh. He was on hemodialysis and had a benign monoclonal gammopathy. Cutaneous biopsy revealed RAE characterized by the proliferation of epithelioid and spindle-shaped cells in superficial and middermis lining vascular channels, arranged in clusters, and sometimes displaying an intravascular growth pattern. These cells stained for CD31, CD34, and actin. Interestingly, prominent amyloid deposits were found in the wall of some vessels in deep dermis, often causing obstruction of their lumina. The cause of RAE is unknown, but it can be associated with infections, antiphospholipid syndrome, dysglobulinemia, cryoproteinemia, and lower extremities arteritis, and it may occur near arteriovenous fistulas. In this patient, we believe that RAE was caused by obliteration of dermal vessels by amyloid deposits. Indeed, it is thought that RAE could be caused by ischemia secondary to vascular obstruction. This is the first reported patient with RAE associated with amyloid deposits.- - - - - - - - - - ranking = 0.046023980910308keywords = spindle (Clic here for more details about this article) |
6/50. Kaposiform hemangioendothelioma: five patients with cutaneous lesion and long follow-up.Kaposiform hemangioendothelioma is a rare locally aggressive vascular tumor of the skin, deep soft tissue, and bone in children, characterized by infiltrating nodules and sheets of spindle cells, and unmistakable resemblance to Kaposi's sarcoma. More than 60 patients with such tumor have been reported so far, and while many have died as a result of extensive disease and severe coagulopathy, the long-term biologic behavior of this tumor remains undetermined. We describe five patients with kaposiform hemangioendothelioma and a mean follow-up of 19 years, ranging from 8 to 35 years. This report emphasizes on the importance of cutaneous lesions being the most commonly affected site, but also for its clinical diversity. early diagnosis is possible even for a small skin lesion, which may be critical for the treatment of a potentially fatal deep-seated extensive tumor. All five patients are well, and three of them with persistent vascular tumor, which has carried two patients from childhood to adult. Although the behavior of this tumor might have been modified by radiation or interferon in three patients, this series indicates that kaposiform hemangioendothelioma is incapable of metastasis, despite a protracted course of many decades with no tendency for spontaneous regression.- - - - - - - - - - ranking = 0.2keywords = spindle cell, spindle (Clic here for more details about this article) |
7/50. A case of head and neck kaposiform hemangioendothelioma simulating a malignancy on imaging.Kaposiform hemangioendothelioma (KH) is an endothelial-derived spindle cell neoplasm often associated with kasabach-merritt syndrome. Most cases arise in infancy and childhood and are soft-tissue tumors. The tumor displays an appearance between capillary hemangioma and Kaposi's sarcoma. We report a case of KH in a 1-year-old girl involving a mass that showed abnormal enhancement of soft tissue superficial to the right temporal bone with partial destruction of the temporal bone, the temporomandibular joint, mandibular condyle, and occipital bone. The physical finding of a discolored mass led clinicians to consider a hemangiomatous lesion, whereas the radiological picture suggested a more aggressive diagnosis of rhabdomyosarcoma and aggressive fibromatosis.- - - - - - - - - - ranking = 0.2keywords = spindle cell, spindle (Clic here for more details about this article) |
8/50. Congenital composite hemangioendothelioma: case report and reappraisal of the hemangioendothelioma spectrum.BACKGROUND: Composite hemangioendothelioma is the most recently described entity of the hemangioendothelioma (HE) spectrum. To the best of our knowledge, only eight cases of this entity have been hitherto described. All of the previous cases affected adults; local recurrences were observed in three cases, and one case showed lymph node metastasis. We herein describe a new and previously unreported congenital case of this rare vascular tumor, arising on the acral extremity of the left forearm, which was diagnosed when the patient was 23 years old. RESULTS: The histological examination disclosed a heterogeneous vascular neoplasm composed of retiform HE-like (80%), spindle cell hemangioma-like (15%), cavernous hemangioma-like (approximately 3%), epithelioid HE-like (approximately 2%) areas, and rare foci with an angiosarcoma-like pattern (< 1%). A distinctive and unique finding of the present case was the presence of large granular eosinophilic macrophages filling some vessels of the retiform HE-like areas. A below-elbow amputation was performed. The patient is alive and well, without evidence of residual or metastatic disease 7 years after the treatment. CONCLUSIONS: The authors expand the concept of composite hemangioendothelioma by adding a congenital case and provide a reappraisal of the hemangioendothelioma spectrum.- - - - - - - - - - ranking = 0.2keywords = spindle cell, spindle (Clic here for more details about this article) |
9/50. Kaposiform hemangioendothelioma of the external auditory canal in an adult.BACKGROUND: Kaposiform hemangioendothelioma is an uncommon vascular tumor initially reported to occur exclusively in children. methods: The presentation, pathologic evaluation, and management of an unusual case of kaposiform hemangioendothelioma is presented and discussed. RESULTS: A 27-year-old hiv-negative man was initially seen with a reddish nodule located in the outer third of the external auditory canal. Histologically, the tumor was composed of spindle-shaped cells arranged in short fascicles associated with small endothelial-like vascular spaces, similar in appearance to Kaposi's sarcoma. The lesion was locally excised but recurred 1 month later; then radiation therapy was performed. The patient remains well at 5-year follow-up. CONCLUSIONS: Recognition of kaposiform hemangioendothelioma is important to avoid possible confusion with a variety of vascular neoplasms with different biologic potential. This case presented some diagnostic difficulty because of the age of the patient and the unusual location of the lesion and had to be mainly distinguished from Kaposi's sarcoma.- - - - - - - - - - ranking = 0.046023980910308keywords = spindle (Clic here for more details about this article) |
10/50. hemangioendothelioma of liver and spleen: trauma-induced consumptive coagulopathy.The case of a 9-year-old boy with hemangioendothelioma of the liver and spleen who presented with consumptive coagulopathy one month after sustaining a blunt trauma to his abdomen is reported. A contrast enhanced computed tomography scan of the abdomen showed a ruptured spleen with multiple lesions in the liver that were enhancing with contrast. On exploration, the child was found to have splenic rupture with multiple vascular lesions of the liver. A splenectomy with liver biopsy was done. The histopathologic examination found that both the liver and spleen had a similar tumor morphology characteristic of an epithelioid and spindle cell hemangioendothelioma. The child ultimately died of relentless consumptive coagulopathy.- - - - - - - - - - ranking = 0.2keywords = spindle cell, spindle (Clic here for more details about this article) |
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