Primary colonic melanoma presenting as ileocecal intussusception: Case report and literature review

Primary malignant melanoma originating in the colon is an extremely rare disease. Herein, we report a case of primary melanoma of the ascending colon. The patient was a 57-year-old male who was admitted to our hospital for persistent abdominal pain and episodes of bloody stool, nausea and vomiting. A computed tomography scan revealed lower intestinal intussusception and enlarged lymph nodes in the abdominal cavity and retroperitoneum. During laparoscopic operation, multiple enlarged lymph nodes were found. Several segments of the proximal small intestine were incarcerated into the distal small intestine, forming an internal hernia and obstruction. The necrotic terminal ileum was invaginated into the ascending cecum. Subsequently, adhesive internal hernia reduction and palliative right hemicolectomy were performed. Pathologic examination of the excised specimen revealed a polypoid mass in the ascending colon. Histological examination showed epithelioid and spindle tumor cells with obvious cytoplasmic melanin deposition. Immunohistochemical staining revealed that the tumor cells were positive for S-100, HMB-45 and vimentin, confirming the diagnosis of melanoma. The patient history and a thorough postoperative investigation excluded the preexistence or coexistence of a primary lesion elsewhere in the skin, anus or oculus or at other sites. Thus, we consider our case to represent an aggressive primary colon melanoma presenting as ileocecal intussusception and intestinal obstruction.     

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.     

Liver abscess complicating Crohn's disease presenting as thoracic empyema. Case report and review of the literature

A 28-year-old man with a history of Crohn's disease presented with right pleuritic pain and dyspnea. Chest radiography showed a right pleural effusion. Thoracocentesis yielded purulent fluid that subsequently grew Enterobacter aerogenes. Computed axial tomography of the abdomen revealed right subphrenic abscess and right hepatic lobe abscess. Antibiotic therapy and surgical drainage resulted in complete recovery. A review of the English literature produced 18 cases of liver abscess complicating Crohn's disease. Details of 14 of these cases are summarized.     

Sarcoidosis presenting as a tumorlike muscular lesion. Case report and review of the literature

Sarcoid myopathy presenting as a tumorlike lesion is an exceedingly rare presentation of sarcoidosis. Concurrent extramuscular involvement is common. Chest radiographs, if abnormal, may suggest the diagnosis. Magnetic resonance imaging is the preferred study for diagnosis and follow-up of tumorous sarcoid myopathy. Optimal therapy is not clear. Favorable responses have been cited with surgery or corticosteroids (alone or in combination). Azathioprine or alternative immunosuppressive agents (for example, antimalarials or methotrexate) may have a role in corticosteroid-recalcitrant patients. The role of local radiotherapy is controversial and should be reserved for severe localized disease refractory to aggressive medical therapy.     

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.     

Erdheim-chester disease. Case report and review of the literature

Erdheim-Chester disease is a distinctive pathologic and radiographic entity characterized by bilateral, symmetric sclerosis of the metaphyseal regions of long bones and infiltration of foamy, lipid-laden histiocytes. Clinically, it ranges from an asymptomatic, focal process to a fatal, systemic disease. All previously reported cases of ErdheimChester disease are reviewed and a new case with diabetes insipidus, partial hypopituitarism, histiocytic skin lesions, and retro-orbital tumors is presented. An 11-year follow-up reveals progression from a benign, limited process to a progressive, systemic disease. Patients with Erdheim-Chester disease show markedly disparate clinical courses and some features similar to Hand-Schüller-Christian disease. They need careful monitoring and further attempts at treatment.     

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.     

Non-Hodgkin's lymphoma presenting as an endobronchial tumor: report of eight cases and literature review

Non-Hodgkin's lymphoma (NHL) involving the endobronchial tree is uncommon, and the initial presentation of NHL as an endobronchial tumor is extremely rare. In a series of 441 patients with newly diagnosed non-Hodgkin's lymphoma over a 7-year period, we reviewed the clinical features of eight patients who presented with an endobronchial tumor. All patients had local pulmonary disease without extrathoracic involvement. The major presenting symptoms were dyspnea, chest pain, cough, and hoarseness. None of the patients had systemic symptoms. Radiographs revealed lobar collapse in all cases. Five patients had mediastinal masses and three had isolated endobronchial lesions. Although MALT lymphoma is the most common primary pulmonary lymphoma, it was present in only one of our patients, while seven patients had aggressive lymphoma. All patients received chemotherapy. Six of the eight patients responded favorably to treatment with complete remission. The prognosis of patients with isolated endobronchial lymphoma is not worse than other local presentations of lymphoma. Bronchoscopic examination with biopsy is essential to differentiate these lesions from primary bronchongenic carcinoma.     

Primary antiphospholipid antibody syndrome presenting with adrenal insufficiency in a child: case report and review of literature

A 7-year-old boy presented with adrenal insufficiency. He subsequently developed venous thrombosis in the limbs and was diagnosed with primary antiphospholipid syndrome (PAPS) based on clinical and laboratory parameters. Both adrenals were normal on imaging. He required thrombolysis and anticoagulation. The progressive course of PAPS was controlled with methylprednisolone. There are few reports of PAPS in pediatric patients, and associated adrenal involvement is rare. The unusual presentation, course and management of the patient and of four other reported children with adrenal insufficiency heralding manifestation of PAPS are discussed.     

Dermatomyositis and Hodgkin's disease. Case report and review of the literature

The association between dermatomyositis and Hodgkin's disease has been infrequently reported. A patient with advanced Hodgkin's disease and dermatomyositis is presented and an additional 11 cases obtained from the world literature are reviewed. The clinical features, response to treatment, and outcome of these diseases are discussed.     

Primary hyperparathyroidism presenting as recurrent acute pancreatitis: A case report and review of literature

The association between pancreatitis and primary hyperparathyroidism (PHPT) is controversial. We report a 32-year-old man who presented with recurrent episodes of acute pancreatitis. Primary hyperparathyroidism was diagnosed after the fourth episode of pancreatitis. He had no additional risk factors for pancreatitis. Eighteen months after successful parathyroid surgery, there has been no recurrence of abdominal pain and his serum calcium is within the normal range.     

Primary hepatic lymphoma: a case report and review of the literature

We report here a case of an older woman, 90 years old on admission, who presented with general deterioration, fever, abdominal pain, large hepatic mass, and was found to have an extra-nodal large B-cell lymphoma of the liver. The patient was successfully treated with multi-agent chemotherapy and followed up for 2 years with no recurrence of the disease. To the best of our knowledge this is the oldest patient reported with such a primary extra-nodal hepatic lymphoma and a remarkably favourable response to chemotherapy.     

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.     

Plasmablastic lymphoma of the small intestine: Case report and literature review

Plasmablastic lymphoma (PBL) is a rare aggressive B-cell lymphoproliferative disorder, which has been characterized by the World Health Organization as a new entity. Although PBL is most commonly seen in the oral cavity of human immunodeficiency virus (HIV)-positive patients, it can also be seen in extra-oral sites in immunocompromised patients who are HIV-negative. Here we present a rare case of PBL of the small intestine in a 55-year-old HIV-negative male. Histopathological examination of the excisional lesion showed a large cell lymphoma with plasmacytic differentiation diffusely infiltrating the small intestine and involving the surrounding organs. The neoplastic cells were diffusely positive for CD79a, CD138 and CD10 and partly positive for CD38 and epithelial membrane antigen. Approximately 80% of the tumor cells were positive for Ki-67. A monoclonal rearrangement of the kappa light chain gene was demonstrated. The patient died approximately 1.5 mo after diagnosis in spite of receiving two courses of the CHOP chemotherapy regimen. In a review of the literature, this is the first case report of PBL with initial presentation in the small intestine without HIV and Epstein-Barr virus infection, and a history of hepatitis B virus infection and radiotherapy probably led to the iatrogenic immunocompromised state.     

Clinically silent primary adrenal lymphoma: A case report and review of the literature

Primary adrenal lymphoma (PAL) is extremely uncommon. We describe a case of clinically silent non-Hodgkin's B-cell lymphoma of diffuse large cell type with exclusive left adrenal localization. The tumor was discovered by computed tomography (CT) as a 2.5-cm dense mass and diagnosed at autopsy. Literature concerning this unusual neoplasm is reviewed. During the early stage, particularly when the lesion is small, PAL is likely to be missed. This unusual entity should be included in the differential diagnosis of adrenal masses so that early diagnosis may be made and intervention might dramatically affect the clinical outcome. Am. J. Hematol. 58:130–136, 1998. © 1998 Wiley-Liss, Inc.     

Pituitary abscess presenting with cranial nerve paresis. Case report and review of literature

Non-adenomatosus lesions of the pituitary represent a small part of the intrasellar processes and they have heterogeneous presentation. Making a precise diagnosis is of great importance, as it may lead to more efficient management. A 65-year-old man was admitted to the hospital because of headache and right cranial nerve III palsy. Basic laboratory work-up was normal whereas endocrinological assessment revealed hypopituitarism without diabetes insipidus. Plain radiography showed an enlarged sella and frontal and paranasal sinusitis. Computed tomography (CT) and magnetic resonance imaging (MRI) of the sella revealed an intrasellar lesion with extension to the sphenoid and cavernous sinuses as well as the suprasellar region, exerting pressure on the optic chiasm. On T1-weighted images the mass had a low-intensity signal with a smooth enhancing rim with bright signal. Given the presence of multiple sinusitis and imaging characteristics a pre-operative diagnosis of pituitary abscess was made. The patient was operated via transphenoidal route and purulent material was drained out. Cultures of the material were positive for Staphylococcus aureus. Antibiotics as well as cortisol replacement therapy were given. Three months later hypopituitarism persisted but there was significant improvement in the neurological findings. We report a case of an unusual presentation of a pituitary abscess. High index of suspicion, the presence of associated conditions such as pituitary tumors, meningitis or sinusitis, as well as diabetes insipidus and specific imaging features are the main diagnostic clues. Pre-operative diagnosis, which will lead to prompt antibiotic therapy and transphenoidal drainage, can decrease high mortality and morbidity associated with this disease.     

Temporal arteritis and renal disease. Case report and review of the literature

Renal abnormalities have been described in a small percentage of patients with temporal arteritis. Transient microscopic hematuria is the most common finding. In rare instances, widespread vasculitis involving renal arteries or microscopic polyarteritis nodosa can be seen. This case report describes the association of temporal arteritis with membranous glomerulonephropathy and the nephrotic syndrome in an elderly patient, an occurrence not previously reported. Whether this association is coincidental or pathogenetically linked remains to be determined.