Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.2.1.17 (lysozyme)
 21,489 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Here, we report an unusual case of gastric anaplastic large cell lymphoma (ALCL), lymphohistiocytic variant, in a 70-year-old female patient who presented with epigastric pain, tarry stool and body weight loss. Endoscopic and imaging findings revealed a Bormann type II tumor in the stomach with perigastric lymphadenopathy and multiple tumor nodules in the liver. Total gastrectomy and liver biopsy were performed. Histologically, both gastric and hepatic tumors demonstrated anaplastic large neoplastic cells scattered among numerous reactive histiocytes. Immunostaining of these tumor cells reacted positively for CD30, CD3, CD45 RO/UCHL1, and negatively for epithelial membrane antigen, CD68, lysozyme, CD15, CD79a, CD138, PAX5 and anaplastic lymphoma kinase. Both the morphologic and immunophenotypic findings supported the diagnosis of gastric ALCL of lymphohistiocytic variant with liver metastasis. This patient then received chemotherapy and was still alive after 17 months of follow-up, without evidence of residual disease.
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PMID:Primary lymphohistiocytic variant of anaplastic large cell lymphoma of the stomach. 1733 48

Sarcoidosis is a granulomatous disease of unknown origin, with pulmonary findings in more than 90% of patients. Extrapulmonary involvement is common and all organs can be involved (especially lymph nodes, eyes, joints, central nervous system) but it is rare to find an isolated extrapulmonary disease (less than 10% of patients). Granulomatous inflammation of the spleen and the liver is common in patients with systemic sarcoidosis, while hepatosplenic enlargement is unusual and splenic involvement rare. We report two cases of systemic sarcoidosis, that onset with splenic and hepatosplenic disease, and one case with splenic sarcoidosis without pulmonary involvement. In the first case a 53-year-old woman with mild abdominal pain underwent sonography and CT, which revealed one hypoechoic/hypodense splenic lesion. Laboratory tests were normal. In order to exclude a lymphoma, splenectomy was performed: histology revealed a sarcoid granuloma. After surgery the patient was asymptomatic and now, after two years, disease is silent. The second case is a 66-year-old woman with a recent weight loss (8 kg in two months) and alterated liver function tests (AST 61 U/l, ALT 72 U/l, Alkaline phosphatase 748 U/l, g-GT 381 U/l). Since she had a familiar history of colon cancer, abdominal US scan, abdominal CT scan and MRI were performed and showed inter-aorto-caval lymphadenopathies and discreet multiple bilobar hepatic and splenic substitutive lesions, with no signs of primary tumor. Upper and lower GI endoscopy, full gynecological workup, complete set of tumor markers, bone marrow biopsy were performed. All resulted negative for neoplasia. Small pulmonary infiltrations were observed on chest-CT scan but cytology on BAL was normal. Infections were also excluded. An exploratory laparotomy showed whitish peritoneal, hepatic and splenic nodules. The histological exam revealed chronic granulomatous lesions typical for sarcoidosis. During a two-year follow-up after the splenectomy the patient feels well without any treatment. The third patient is a 32-year-old woman with mild epigastric pain after meals. Neck-thoracic CT, bone scintigraphy and upper GI endoscopy were negative. Abdominal US and MR showed splenomegaly with multiple splenic lesions. Splenectomy was performed and histological exam showed chronic granulomatous lesions typical for sarcoidosis. Further laboratory tests were normal, except for ACE (66 UI/l). After the surgery ACE became normal and now, three years later, the patient is still asymptomatic. We conclude that hepatosplenic involvement is less rare than it is thought. It is often oligosymptomatic or accompanied with unspecific manifestations and laboratory abnormalities. The diagnosis could be difficult; in fact typical laboratory findings of sarcoidosis such as ACE, lysozyme, calcium, were not diagnostic. Ultrasonography and CT were important but the diagnosis was established only with the histological examination of suspected lesions. This latter required to differentiate liver and/or spleen sarcoidosis from tuberculosis and other infections, primary biliary cirrhosis, metastasis or malignant lymphoma.
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PMID:Atypical sarcoidosis: case reports and review of the literature. 2138 7

Histiocytic sarcoma is a rare malignant neoplasm that originates from a histiocytic hematopoietic lineage characterized by histiocytic differentiation and its corresponding immunophenotypic features. Patients with histiocytic sarcoma usually have a poor prognosis due to its aggressive clinical behavior. Here we report a rare case of extranodal histiocytic sarcoma of the stomach which was confirmed through immunohistochemical staining. A 71-year- old man was presented with epigastric pain. Gastroscopy, abdominal CT, and EUS revealed a mass located on the posterior wall of upper body and fundus of the stomach. Grossly, grayish white solid masses were seen extending down to the submucosal layer. Microscopically, the tumor cells had eosinophilic cytoplasm, abundant vacuole, and mitosis. Immunohistochemical staining revealed that the tumor cells were positive for LCA, CD68, and lysozyme. Early detection and accurate diagnosis of this rare neoplasm is important because it can make a great difference in prognostic outcomes. To make an accurate and definitive diagnosis, immunohistochemical staining is essential in the confirmation of histiocytic origin.
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PMID:[A case of extranodal histiocytic sarcoma of stomach mimicking gastric adenocarcinoma]. 2016 59

Histiocytic sarcoma is a rare lymphohematopoietic malignancy with aggressive clinical course and poor therapy response. The diagnosis relies on the confirmation of its histiocytic lineage and exclusion of other poorly differentiated tumors. Most of the cases present in extranodal sites, but primary gastric involvement is exceptional. We report a case of a 69-year-old woman with epigastric pain and systemic symptoms. Gastroscopy findings and biopsy report suggested a malignant neoplasm. The patient underwent distal subtotal gastrectomy with a 6-cm tumor in the body and antrum of the stomach and ten associated enlarged perigastric lymph nodes. Microscopically they were infiltrated with atypical tumor cells and immunohistochemical staining was positive for CD68, lysozyme, CD45, and CD4; 45% of the cells stained for Ki-67. The pathologic diagnosis was histiocytic sarcoma. CT body scans showed only enlarged retroperitoneal and abdominal lymph nodes. The patient received six cycles of CHOEP chemotherapy with complete therapeutic response, but three months later she experienced an aggressive systemic sarcoma recurrence and although salvage chemotherapy was initiated she died of progressive disease. The presented case widens the differential diagnosis of gastric malignancies, and emphasizes the significance of immunohistochemical examination for histiocytic sarcoma diagnosis. The collection and evaluation of cases of gastric histiocytic sarcoma are important to obtain further progress in prognosis and treatment.
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PMID:A Rare Case of Primary Histiocytic Sarcoma of the Stomach. 3035 26