原发性肝脏T细胞淋巴瘤1例报告并文献复习

原发性肝脏淋巴瘤(primary hepatic lymphoma,PHL)是一种源自肝内淋巴组织的少见恶性肿瘤,迄今报道均为非霍奇金淋巴瘤,国内外文献报道仅100余例,约占结外淋巴瘤的0.14％,占肝脏恶性肿瘤的0.11％[1].国外报道部分PHL患者可合并自身免疫性疾病、慢性肝炎、原发性胆汁性肝硬化、乙型肝炎病毒(hepatitis B virus,HBV)和丙型肝炎病毒(hepatitis C virus,HCV)感染以及获得性免疫缺乏综合征(acquired immune deficiencysyndrome,AIDS)等[2].     

Primary adrenal lymphoma: a case report and literature review in Japan

A 78-year-old male was admitted to our hospital complaining of nausea, general fatigue and anorexia in November, 1999. Clinical findings on admission were weight loss and dehydration but surface lymph nodes were not palpable. Masses in the bilateral adrenal glands were detected by ultrasonography, computed tomography and magnetic resonance imaging. Laboratory examinations revealed hyponatremia and hyperkalemia. Subsequent endocrine function tests showed normal serum cortisol and increased adrenocorticotropic hormone (ACTH) levels. Rapid ACTH test and cortico-hormone releasing hormone (CRH) test revealed insufficient secretion of cortisol. The histological diagnosis of the adrenal gland by laparotomy was diffuse large B-cell lymphoma. We diagnosed primary adrenal lymphoma with adrenal insufficiency. The patient underwent hormone supplementary therapy and chemotherapy, but he died two months later. We report on this rare primary adrenal lymphoma case and summarize the reports of this disease in the Japanese literature.     

Primary hepatic carcinoid： A case report and literature review

Carcinoids are tumors derived from neuroendocrine cells and often produce functional peptide hormones. Approximately 54.5% arise in the gastrointestinal tract and frequently metastasize to the liver. Primary hepatic carcinoid tumors （PHCT） are extremely rare;only 95 cases have been reported. A 65-year-old man came to our attention due to occasional ultrasound findings in absence of clinical manifestations. His previous medical history, since 2003, included an echotomography of the dishomogeneous parenchymal area but no focal lesions. A computed tomography scan performed in 2005 showed an enhanced pseudonodular-like lesion of about 2 cm. Cholangiomagnetic resonance imaging identified the lesion as a possible cholangiocarcinoma. No positive findings were obtained with positron emission tomography.Histology suggested a secondary localization in the liver caused by a low-grade malignant neuroendocrine tumor. Immunohistochemistry was positive for anti chromogranin antibodies, Ki67 antibodies and synaptophysin. Octreoscan scintigraphy indicated intense activity in the lesion. Endoscopic investigations were performed to exclude the presence of extrahepatic neoplasms. Diagnosis of PHCT was established.The patient underwent left hepatectomy, followed by hormone therapy with sandostatine LAR. Two months after surgery he had a lymph nodal relapse along the celiac trunk and caudate lobe, which was histologically confirmed. The postoperative clinical course was uneventful, with a negative follow-up for hematochemical, clinical and radiological investigations at 18 mo post-surgery. Diagnosis of PHCT is based principally on the histopathological confirmation of a carcinoid tumor and the exclusion of a non-hepatic primary tumor. Surgical resection is the recommended primary treatment for PHCT. Recurrence rate and survival rate in patients treated with resection were 18% and 74%, respectively.     

Primary hepatic non-Hodgkin's lymphomas: case report and review of the literature

A 31-yr-old man presented with a 1-wk history of fever, chills, weakness, headaches, and a significant 20-lb weight loss over the preceding 2 months. His past medical history was relevant for liver amebiasis during childhood. Two days before admission, the patient noticed jaundice. He denied abdominal pain or other GI symptoms, and there was no history of alcohol intake, medications, or illicit drugs. His physical examination revealed generalized jaundice, hepatosplenomegaly, and bilateral leg edema. Neurologically, the patient was agitated, with periods of disorientation, and he had bilateral flapping. His blood tests revealed pancytopenia, renal failure, liver failure, and coagulopathy. Because the patient had a fever, hepatosplenomegaly, and pancytopenia, a further workup also included a bone marrow and liver biopsy. No conclusive diagnosis could be made from the above tests, and the patient died 5 days after admission. Postmortem evaluation, including flow cytometry and gene rearrangement in the tissue obtained from the liver, revealed large B cell lymphoma. This case illustrates an unusual presentation of hepatic non-Hodgkin's lymphoma. Current information regarding this entity is scant, mainly owing to its rarity. We present a review of the literature, including the incidence, presentation, treatment, and prognosis of primary hepatic lymphoma.     

Primary hepatic amyloidosis: A case report and review of literature

We describe a case of 42-year-old female presenting with abdominal pain associated with loss of weight and fever for 8 mo. On evaluation she had gross hepatomegaly with raised alkaline phosphatase and raised GGT levels with normal transaminases and bilirubin. On imaging she had diffuse enlargement of liver with heterogeneous contrast uptake in liver. Her viral marker and autoimmune markers were negative. Liver biopsy depicted massive deposition of amyloid in peri-sinusoidal spaces which revealed apple green birefringence on polarizing microscopy after Congo red staining. Cardiac and renal evaluation was unremarkable. Abdominal fat pad and rectum biopsy was negative for amyloid deposit. There was no evidence of primary amyloidosis as bone marrow examination was normal. Serum and urine immunofixation electrophoresis were normal. Immunoperoxidase staining for serum amyloid associated protein for secondary amyloidosis was negative from liver biopsy. We present this rare case of primary hepatic amyloidosis and review the literature regarding varied presentations of hepatic involvement in amyloidosis.     

Primary extranodal non-Hodgkin's lymphoma of the vagina: a case report and a review of the literature

Primary lymphoma of the female genital tract is very rare. We report the case of a 36-year-old woman who was referred to our hospital because of an indeterminate Pap smear test. The colposcopy showed a thickening of the posterior vaginal wall and various irregular ulcerated nodular lesions. Histological examination, immunohistochemistry and the staging procedures were conclusive of diffuse large B-cell lymphoma of the vagina, stage IEA. Complete remission was achieved after 6 cycles of immunopolychemotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone). No relapse has occurred during a follow-up of 71 months. Moreover, we reviewed the 62 previously reported cases of primary extranodal non-Hodgkin's lymphoma of the vagina, focusing on clinicopathological and therapeutic aspects, to better characterize this unusual disease.     

Primary malignant lymphoma of uterine corpus: case report and review of the literature

We describe a patient presenting with postmenopausal vaginal bleeding and a uterine mass subjected to endometrial biopsy that showed a high-grade non-Hodgkin's lymphoma, consistent with a diffuse large B-cell lymphoma. Staging computed tomography (CT) scans of the chest, abdomen, and pelvis revealed three lung nodules in addition to the uterine mass. Fine needle aspirate of one lung lesion showed lymphomatous involvement. She was treated with intensive chemotherapy alone and has remained in complete remission 21 months after diagnosis. The literature on primary lymphoma of the uterine corpus is reviewed.     

Primary low-grade B-cell lymphoma of the urinary bladder: case report and literature review

The first recorded case of lymphoma of the bladder was reported by Eve and Chaffey in 1885. Malignant lymphoma of the bladder can be classified into one of three different clinical groups: 1) Primary lymphoma localized to the bladder; 2) Lymphoma presenting in the bladder as the first sign of disseminated disease (non-localized lymphoma); 3) Recurrent bladder involvement by lymphoma in patients with a history of malignant lymphoma (secondary lymphoma). Primary extranodal marginal zone lymphoma of mucosa associated lymphoid tissue (MALT type) of the urinary bladder, first described by Kempton et al in 1990, is the most common primary bladder lymphoma and associated with an excellent prognosis. We present a patient with gross hematuria who was found to have a primary bladder lymphoma and review the relevant literature.     

Primary anaplastic large cell lymphoma of the psoas muscle: a case report and literature review

Primary anaplastic large cell lymphoma (ALCL) of skeletal muscle is very rare. We report a case of ALCL arising from the left psoas muscle. A 14-year-old girl presented with a large left inguinal tumor. She complained of a 2-month history of left leg pain, which had been exacerbated upon leg extension, and she had become aware of a rapidly growing left inguinal tumor 3 weeks before admission. CT scan and MRI revealed a large tumor arising from the left major psoas muscle and protruding into the inguinal region. In view of the tumor's location and the patient's age, soft tissue tumors such as rhabdomyosarcoma and primitive neuroectodermal tumor were initially considered. However, histopathological examination yielded a diagnosis of anaplastic lymphoma kinase-positive ALCL. The serum level of soluble interleukin-2 receptor was markedly elevated at 50,414 U/ml, and this also strongly suggested ALCL. Although rarely reported, ALCL is an important entity to consider in the differential diagnosis of skeletal muscle tumors in children and young adults.     

Primary adrenal adult T-cell leukemia/lymphoma: a case report and review of the literature

Primary adrenal lymphoma (PAL) is very rare; the majority of cases reported previously were of B-cell origin. We report a rare case of primary adrenal adult T-cell leukemia/lymphoma (primary adrenal ATLL). ATLL is a highly aggressive T-cell type non-Hodgkin's lymphoma and etiologically associated with human T-cell lymphotropic virus 1 (HTLV-1). Most ATLL patients present with leukemia and widespread lymphadenopathy. A 37-year-old Japanese woman presented with back pain in January 2004. Examination showed no peripheral lymphadenopathy, circulating lymphoma cells, hepatosplenomegaly, and skin lesions. Imaging studies demonstrated large adrenal masses bilaterally. Subsequently, she underwent open adrenal biopsy and pathological diagnosis was confirmed as T-cell lymphoma. The serum antibody to HTLV-1 was positive. Southern blot analysis detected monoclonal integration of proviral DNA of HTLV-1 into host genome in the biopsy specimen. The diagnosis of ATLL arising in adrenal glands was established. Despite repeated systemic chemotherapy, the patient died of progressive disease in December 2004. ATLL could primarily involve the adrenal gland and this disease entity should be included in the differential diagnosis of adrenal mass lesions.     

Primary non-Hodgkin's lymphoma of the vagina case report and review of the literature

The genital tract as primary site of malignant non-Hodgkin's lymphoma in women is extremely rare, whereas secondary involvement in advanced disease is found in about 40% of cases. In this report a patient is presented who had a primary vaginal non-Hodgkin's lymphoma of the centroblastic type according to the Kiel classification, with an excellent response to cytotoxic chemotherapy (CHOP) and event-free disease for 3 years. A review of the literature shows that favorable prognosis of localized disease seems to be a common experience. Primary involvement of the vagina can be successfully treated by pelvic irradiation, but in young women cytotoxic chemotherapy should be considered to preserve fertility.     

Primary hepatic marginal zone B cell lymphoma of mucosa-associated lymphoid tissue type: case report and review of the literature

A primary hepatic marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT) is very rare. We found a solitary mass 27 mm in size in the left lobe of the liver of a 58-year-old Japanese man with a history of hepatitis-C infection. Based on the results of imaging studies, the tumor was diagnosed as a hepatocellular carcinoma (HCC). The left lobe of the liver was lobectomized and microscopic findings showed that the tumor was a hepatic MALT lymphoma, while immunohistochemistry showed it to be positive for CD20 and CD79a. In a fluorodeoxyglucose-positron emission tomography examination integrated with computed tomography scanning (FDG-PET CT) before surgery, the tumor was revealed to have a high standardized uptake value (SUV) for FDG. The patient received chemotherapy after surgery. To the best of our knowledge, 45 cases had been reported with a mean age for all patients of 61.4 years. The pathogenesis remains unclear, although half of the patients had a past history of chronic inflammatory liver disease. Surgical resection was performed in most cases and some patients received postoperative chemotherapy or radiotherapy. The clinicopathologic characteristics and management of this extremely rare disease are also discussed.     

Primary hepatic signet ring cell neuroendocrine tumor: a case report with literature review

Primary hepatic signet ring cell neuroendocrine tumor is extremely rare and is characterized by distinct intracytoplasmic hyaline vacuoles that are mucin negative and cytokeratin positive. The unique histological features may cause difficulty in diagnosis and delay patient care. Here the authors report a 49-year-old man with an incidental finding of a 2.7 cm liver mass in the absence of chronic liver disease. The resected tumor was grossly unencapsulated but well demarcated with friable tissue texture. Microscopically, the entire tumor consisted of sheets of monotonous cells separated by delicate microvasculature. The tumor cells had granular chromatin, inconspicuous nucleoli, and eosinophilic cytoplasm. Many of the tumor cells had eccentric, pale intracytoplasmic vacuoles resembling signet ring cells in adenocarcinoma. Immunohistochemical studies showed that the tumor cells were positive for neuroendocrine markers and that the intracytoplasmic vacuoles were negative for mucin but strongly positive for cytokeratins. Careful systemic search including OctreoScan scintigraphy (Mallinckrodt Medical, Inc., St. Louis, MO) and capsule endoscopy failed to reveal any other tumors. A diagnosis of primary hepatic signet ring cell neuroendocrine tumor was established. Ten months after surgery, the patient is well without any other detectable tumor on radiology. Serological neuroendocrine markers are also within normal limits.     

Primary cardiac lymphoma and complete atrio-ventricular block: case report and review of the literature

Primary cardiac lymphoma (PCL) is a rare and extremely aggressive malignancy, which can express itself by damaging the cardiac conduction system (complete atrio-ventricular block), the myocardium and the pericardium. Histology confirms the diagnosis. Chances of survival depend on early diagnosis and treatment. We present the case of a female patient admitted for severe deterioration of her general state of health who had a complete atrio-ventricular block caused by a tumour of the atrial septum, a B-cell lymphoma stage I. The diagnosis was suggested by combined PET/CT imaging and confirmed by histology of a cardiac biopsy. Her general clinical condition, including the cardiac parameters, significantly improved following chemotherapy induction.     

Primary T-cell pulmonary lymphoma. A case report and review of the literature

Non-Hodgkin's lymphomas rarely involve the pulmonary parenchyma. Most cases described to date involve B-cells. We report the first case of T-cell pulmonary lymphoma in the Spanish literature. The course of disease was rapid and response to treatment was poor. Such cases demonstrate the importance of open biopsy and the study of cell markers for diagnosis. T-cells lymphomas should be included in the differential diagnosis of febrile patients with pulmonary nodules.