A 62-year-old woman was evaluated for tinnitis and headache. Magnetic resonance imaging and angiography revealed the coexistence of a tentorial tumor encroaching the junction of the right transverse-sigmoid sinuses, and dural arteriovenous fistulous malformation (AVFM) of the right transverse sinus. AVFM was not manipulated at all during the surgery. The pathology was fibroblastic meningioma. Postoperatively, the dural AVFM completely disappeared on follow-up angiography. The fistulas were occluded also after surgery, even though there was no manipulation of the AVFM. It is suggested that the right dominant transverse-sigmoid sinuses are partially occluded by tentorial meningioma, developing the dural arteriovenous fistula of the right transverse sinus. An acquired origin of the dural AVFM was suggested in this case.
Arteriovenous Malformations/diagnosis ; Arteriovenous Malformations/complications* ; Carotid Artery, External/pathology ; Carotid Artery, Internal/pathology ; Case Report ; Cerebral Angiography ; Dura Mater/pathology ; Female ; Human ; Jugular Veins/pathology ; Magnetic Resonance Imaging ; Meningeal Neoplasms/diagnosis ; Meningeal Neoplasms/complications* ; Meningioma/diagnosis ; Meningioma/complications* ; Middle Age

Mantle cell lymphoma (MCL) is an uncommon type of gastrointestinal lymphoma. MCL is a distinct subtype of B-cell non-Hodgkin lymphomas. The major subtype of MCL is characterized by the presence of multiple lymphomatous polyposis (MLP), in which multiple polyps are observed along the gastrointestinal tract. The malignant cells express pan B-cell marker and the T-cell marker cluster of differentiation 5. The chromosomal translocation t(11;14)(q13;q32) that causes cyclin D1 overexpression is commonly observed on the cytogenetic analysis of MCL. Survival improvement has recently been achieved for patient with MCL by the successful introduction of monoclonal antibodies and dose-intensified approaches for treatment, including autologous stem cell transplantation strategies. Some reports suggest that there is an increased incidence of second malignancies in patients with MCL or lymphoma. We report a case of MCL involving the colon; the patient was a 60-year-old man who complained of low abdominal discomfort during defecation. During the workup, a meningioma was unexpectedly discovered. On analysis, the tumor was found to be a t(11;14)-negative and non-MLP-type MCL.
Chromosomes, Human, Pair 11 ; Chromosomes, Human, Pair 14 ; Cyclin D1/metabolism ; Humans ; Lymphoma, Mantle-Cell/*diagnosis/genetics/metabolism ; Magnetic Resonance Imaging ; Male ; Meningeal Neoplasms/complications/*diagnosis/pathology ; Meningioma/complications/*diagnosis/pathology ; Middle Aged ; Positron-Emission Tomography ; Translocation, Genetic

Multiple endocrine neoplasia type 1 (MEN1) syndrome includes varying combinations of endocrine and non-endocrine tumors. There are also a considerable number of atypical MEN1 syndrome. In this case, a 68-yr-old woman was referred to the Department of Endocrinology for hypercalcemia. Five years ago, she had diagnosed as primary hyperaldosteronism and now newly diagnosed as parathyroid hyperplasia with laboratory and pathologic findings. Hurthle-cell thyroid cancer was also resected during the parathyroid exploration and small meningioma was found on brain MRI. Her general condition has markedly improved and her adrenal mass and meningioma are being closely observed now. We could find the loss of heterozygosity of the MEN1 locus in parathyroid glands, suggesting a MEN1-related tumor, but not a germline mutation. Considering a variety of phenotypic expression and a limitation of current molecular analysis, periodic follow up will be needed in patients with a MEN1-like phenotype.
Aged ; Base Sequence ; Brain/radionuclide imaging ; Female ; Humans ; Hyperaldosteronism/complications/*diagnosis ; Hyperparathyroidism, Primary/*diagnosis/etiology/pathology ; Loss of Heterozygosity ; Magnetic Resonance Imaging ; Meningeal Neoplasms/complications/*diagnosis/radionuclide imaging ; Meningioma/complications/*diagnosis/radionuclide imaging ; Mutation ; Parathyroid Glands/pathology ; Proto-Oncogene Proteins/genetics/metabolism ; Sequence Analysis, DNA ; Thyroid Neoplasms/complications/*diagnosis/pathology ; Tomography, X-Ray Computed

OBJECTIVETo study the clinicopathologic features of follicular dendritic cell sarcoma (FDCS) and its differential diagnosis.

METHODSTen cases of FDCS were studied by light microscopy, immunohistochemistry and in-situ hybridization. The clinical features and follow-up information were analyzed.

RESULTSAmongst the 10 cases of FDCS studied, the male-to-female ratio was 1:1. The mean age of the patients was 42 years. Six of them were located in cervical and peritoneal lymph nodes and four in extranodal sites (including tonsil, pelvic cavity, tail of pancreas and spleen). Histologically, the tumor cells had whorled, storiform or diffuse growth patterns. They were spindle in shape and contained syncytial eosinophilic cytoplasm, with round or oval nuclei, vesicular chromatin, distinct nucleoli and a variable number of mitotic figures. Multinucleated tumor giant cells and intranuclear pseudoinclusions were occasionally seen. There was a sprinkling of small lymphocytes and neutrophils within the tumor as well as in the perivascular region. Immunohistochemical study showed that the tumor cells were diffusely or focally positive for CD21, CD23, CD35 and D2-40, but negative for LCA, CD20, CD3, CD1a, HMB45 and CK. Some of them showed EMA, CD68 and S-100 reactivity. In-situ hybridization for Epstein-Barr virus-encoded RNA (EBER) showed positive signals in only one case (which was diagnosed as inflammatory pseudotumor-like FDCS). Of the 7 patients with follow-up information available (duration: 2 months to 39 months; mean: 14 months), 2 cases with paraneoplastic pemphigus died of pulmonary infection at 5 and 7 months respectively. The remaining 5 patients were alive and disease-free after surgical excision (+/- chemotherapy and radiotherapy).

CONCLUSIONSFDCS is a rare low to intermediate-grade malignant tumor. Appropriate application of FDC markers, such as CD21, CD35 and D2-40, would be helpful for arriving at a correct diagnosis. Most cases are associated with good prognosis after surgical treatment, with or without chemotherapy and radiotherapy. Patients with paraneoplastic pemphigus carry a less favorable prognosis.

Adult ; Antibodies, Monoclonal, Murine-Derived ; metabolism ; Dendritic Cell Sarcoma, Follicular ; complications ; metabolism ; pathology ; surgery ; Dendritic Cell Sarcoma, Interdigitating ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Lymph Node Excision ; Lymph Nodes ; pathology ; surgery ; Male ; Meningioma ; pathology ; Middle Aged ; Nasopharyngeal Neoplasms ; pathology ; Paraneoplastic Syndromes ; complications ; Pemphigus ; complications ; Receptors, Complement 3b ; metabolism ; Receptors, Complement 3d ; metabolism ; Receptors, IgE ; metabolism ; Tonsillar Neoplasms ; metabolism ; pathology ; surgery ; Young Adult