1/201. Systemic granulomatous arteritis associated with Epstein-Barr virus infection.A 61-year-old woman initially presented with symptoms and findings reminiscent of infectious mononucleosis, and her illness then took a rapidly fatal course. autopsy revealed widespread granulomatous arteritis, with multinucleated giant cells but without eosinophils and fibrinoid necrosis, affecting small arteries and arterioles and infiltration of haemophagocytic histiocytes into many organs. in situ hybridization with Epstein-Barr virus (EBV)-specific oligonucleotide probes showed positive signals in the infiltrating immune cells and epithelial and endothelial cells of the affected organs. EBV-associated haemophagocytic syndrome (EBV-AHS) with systemic granulomatous arteritis was diagnosed. From the immunophenotypes of the infiltrating immune cells, a possible role of CD4 T-cells in the pathogenesis of this haemophagocytic syndrome and granulomatous vasculitis was suggested.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
2/201. Double cancers of the lung and esophagus associated with a sarcoid-like reaction in their regional lymph nodes: report of a case.A case of double cancers of the lung and esophagus associated with a sarcoid-like reaction in their regional lymph nodes is reported. A 73-year-old man with hemosputum was found to have a mass in his right lower lung field on a chest X-ray. Based on a diagnosis of lung cancer, a right middle and lower lobectomy with a dissection of the lymph nodes was performed. Microscopically, a well developed granulomatous reaction was seen in the dissected mediastinal and hilar lymph nodes. Three years after the pulmonary resection, he was admitted to our hospital because of dysphagia. A diagnosis of lower esophageal cancer was made. A lower esophagectomy with a total gastrectomy was performed. A sarcoid-like reaction comprising epithelioid cells and giant cells was seen in the regional lymph nodes. No clinical findings indicative of systemic sarcoidosis were observed. This rare condition may therefore help to improve our overall understanding of the relationship between malignant neoplasms and sarcoid-like reactions in the regional lymph nodes.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
3/201. Histopathology and electron and immunofluorescence microscopy of gingivitis granulomatosa associated with glossitis and cheilitis in a case of Anderson-fabry disease.A 17-year-old white boy with signs, symptoms, and family history of angiokeratoma corporis diffusum universale, Anderson-fabry disease (AFD), developed recurrent and then persistent swelling of both lips, erythematous hyperplastic gingivae, and a pebbled tongue. Positive blood findings were raised serum IgE, decreased T-cell level, and increased B-cell level. Histopathology of the gingiva showed noncaseating granulomas with multinucleate giant cells containing Schaumann bodies and large plasma-cell infiltrates in which immunofluorescence demonstrated immune globulins of several classes. Electron microscopy and histochemistry demonstrated ceramide in the vasculature. No glycolipid was found in the macrophages or giant cells of the granulomas which, in contrast, resembled sarcoid reactions. plasma cells with Russell bodies and immune reaction-induced degranulation of mast cells were also identified. The pathogenesis of the oral findings possibly relates to altered immune reactivity associated with damage to the microvasculature analogous to that in melkersson-rosenthal syndrome.- - - - - - - - - - ranking = 2keywords = giant (Clic here for more details about this article) |
4/201. Granulomatous mycosis fungoides: report of a case with some histopathologic features of granulomatous slack skin.We describe a case of granulomatous mycosis fungoides, tumor stage, mimicking sarcoidosis in an 82-year-old man with a 2-year history of skin disease. The final diagnosis was established after one of seven biopsy specimens showed a nongranulomatous histologic picture of patch-stage mycosis fungoides. Monoclonality was proven for the lymphocytic population by T-cell-receptor rearrangement studies. The unusually extensive granulomatous inflammation with huge giant cells surrounded by CD1a-positive cells in the other six biopsy specimens was suggestive of the histopathology of granulomatous slack skin, another rare granulomatous cutaneous T-cell lymphoma. Because both a clinical and histologic overlap between granulomatous mycosis fungoides and granulomatous slack skin have been reported in the literature, we conclude that they may belong to the spectrum of a single disease.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
5/201. Infantile disseminated visceral giant cell arteritis presenting as sudden infant death.The rare clinicopathological entity 'disseminated visceral giant cell arteritis' (DVGCA) was first described in 1978. It is characterized by widespread small-vessel giant cell angitis and extravascular granulomas. A normal and healthy 7-month-old boy who presented unexpectedly with sudden infant death syndrome (SIDS) is reported. Histological examination at autopsy revealed giant cell angitis of the aorta, common carotid, coronary, pulmonary, celiac, mesenteric and common iliac arteries. There were also granulomas in the tracheal wall and liver. To our knowledge, this is the first documented case of DVGCA occurring in an infant younger than 12 months of age. A review of the literature on DVGCA is presented in this report, and the differential diagnosis is discussed.- - - - - - - - - - ranking = 7keywords = giant (Clic here for more details about this article) |
6/201. Carcinoma in villous adenoma of ascending colon associated with sarcoid reaction in the regional lymph nodes.A 79-year-old woman was admitted to our hospital due to continuous anal bleeding. colonoscopy showed a huge villous tumor on the middle area of the ascending colon. A typical right colectomy and lymph node dissection were performed. The resected specimen showed a villous type tumor located on the ascending colon. The histopathologic investigation demonstrated a moderately differentiated adenocarcinoma arising in a tubulovillous adenoma and extending to the submucosa. Although there was no evidence of metastatic carcinoma in the dissected lymph nodes, epithelioid cell granulomas with multinucleated giant cells lacking in the central caseous necrosis suggested sarcoid reaction.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
7/201. Dermatophytic granuloma caused by microsporum canis in a heart-lung recipient.We present a case of dermatophytic granuloma caused by microsporum canis in a heart-lung recipient. This 66-year-old man was seen for erythematous pustules and papules on the forearm. The diagnosis was suspected after histological examination showing an inflammatory infiltrate in the upper dermis with giant cells containing intracytoplasmic fungal elements. Cultures of the skin biopsy confirmed the diagnosis identifying M. canis. Our case emphasizes the possibility of deep dermatophytic infections in immunocompromised patients. There are only 4 additional reports of M. canis infection responsible for invasion of the dermis in such patients. The follicle involvement probably explains these dermal lesions due to the progression of the dermatophyte from the hair follicle to the dermis. In our observation topical antifungal therapy alone was unsuccessful and fluconazole seems to be the treatment of choice for these M. canis invasive dermal cutaneous infections.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
8/201. MR imaging of a hemorrhagic and granulomatous cyst of the ligamentum flavum with pathologic correlation.cysts of the ligamentum flavum are uncommon causes of neurologic signs and symptoms and usually are seen in persons over 50 years of age. We report a case of an epidural cyst located in the ligamentum flavum, which contributed to spinal stenosis in a 30-year-old man. Radiologic features were similar to those of a synovial cyst, but synovium was not identified histologically. The imaging and pathologic features were unusual, including hemorrhage and a fibrohistiocytic reaction with giant cells.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
9/201. A patient with diabetes insipidus and periorbital swellings; erdheim-chester disease.erdheim-chester disease is a rare multisystem disease in which a progressive xanthogranulomatous infiltration of several tissues can be seen. We describe a woman, known to have diabetes insipidus for ten years, with periorbital, retroperitoneal, mediastinal, axillar and inguinal involvement. On histological examination a granulomatous infiltration of fatty tissue and striated muscle was seen, consisting of Touton giant cells, histiocytes with foamy cytoplasm and lymphocytes. Immunohistochemical staining with CD-1a and S-100 was negative and on electron microscopy no Langerhans granules were seen. These findings led to the diagnosis of erdheim-chester disease. She had a good response on steroids. Because of some similar clinical features of Langerhans cell histiocytosis and erdheim-chester disease, a histiocyte disorder seems the most probable cause.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
10/201. liver granulomatosis is not an exceptional cause of hypercalcemia with hypoparathyroidism in dialysis patients.In 4 of our patients on chronic dialysis, we were intrigued by the association of hypercalcemia /- hyperphosphatemia and normal intact PTH, with anicteric cholestasis without cytolysis. This picture occurred in 2 patients after they resumed dialysis because of a transplant rejection and in a third one after discontinuation of corticosteroids, prescribed for an idiopathic thrombocytopenia. No patient was under calcitriol, CaCO3 therapy, and their hypercalcemia persisted on a low calcium dialyzate (1.25 mmol/l). Obvious etiologies of hypercalcemia were not found: vitamin d or A intoxication, hyperparathyroidism, aluminum intoxication, hemopathy, hiv infection. The hypothesis of a granulomatous disease was made and a liver biopsy was performed showing granulomas with giant epitheloid cells. In one case foreign material (silicon ?) was present in the macrophages. Extensive investigations for sarcoidosis, tuberculosis and mycosis were negative. In 2 cases the so-called "dialysis" granulomatosis actually occurred in transplanted patients, suggesting the role of a transplantation related factor (toxic or virus). In the last case HCV seroconversion was present. In the 4 cases, corticotherapy led to the disappearance of hypercalcemia and to an increase of PTH. Our patients had the biological pattern of low bone turnover disease (hypercalcemia and normal intact PTH) and bone biopsy performed in 2 showed osteomalacia or ABD without aluminum. The association of this pattern with cholestasis should evoke liver granulomatosis, which should be confirmed by a liver biopsy and lead to a treatment by corticosteroids. The masking effect of previous corticoid therapy for transplantation should be pointed out. In 2 cases serial monitoring of plasma calcitriol showed a relation between decreasing high normal calcitriol with prednisone and normalization of calcemia, suggesting the role of inappropriate synthesis of calcitriol by the granuloma. In conclusion, liver granulomatosis should be looked for in dialysis patients on the association of unexplained hypercalcemia and normal PTH with anicteric cholestasis, and confirmed by a liver biopsy. Although still of unknown etiology, its evolution is favourable under corticotherapy.- - - - - - - - - - ranking = 1keywords = giant (Clic here for more details about this article) |
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