Mantle cell lymphoma of the prostate

Lymphoma of the prostate, either primary or secondary, is very rare. We report the case of an 82-year-old man with symptoms of bladder outlet obstruction presumably due to benign prostate hyperplasia (BPH). He underwent an uneventful transurethral resection of the prostate. Pathological review of the resected tissue demonstrated substantial infiltration by an atypical lymphoid infiltrate positive for CD20, BCL2, CD5, κ light chain and cycline D1. Histology and immunoprofile were consistent with mantle cell lymphoma.     

Molecular characteristics of mantle cell lymphoma presenting with clonal plasma cell component

The normal counterparts of mantle cell lymphoma (MCL) are naive, quiescent B cells that have not been processed through the germinal center (GC). For this reason, although lymphomas arising from GC or post-GC B cells often exhibit plasmacytic differentiation, MCL rarely presents with plasmacytic features. Seven cases of MCL with a monotypic plasma cell (PC) population were collected from 6 centers and were studied by immunohistochemistry, fluorescence immunophenotyping and interphase cytogenetics as a tool for the investigation of neoplasms analysis, capillary gel electrophoresis, and restriction fragment length polymorphism of immunoglobulin heavy chain analysis of microdissections of each of the MCL and PC populations to assess their clonal relationship. The clinical presentation was rather unusual compared with typical MCL, with 2 cases arising from the extranodal soft tissues of the head. All MCL cases were morphologically and immunohistochemically typical, bearing the t(11;14)(q13;q32). In all cases, the PC population was clonal. In 5 of the 7 cases, the MCL and PC clones showed identical restriction fragments, indicating a common clonal origin of the neoplastic population. The 2 cases with clonal diversity denoted the coexistence of 2 different tumors in a composite lymphoma/PC neoplasm. Our findings suggest that MCL can present with a PC component that is often clonally related to the lymphoma, representing a rare but unique biological variant of this tumor.     

Mantle zone lymphoma. Immuno- and enzymehistochemical studies on the cell of origin

Using in-situ immuno- and enzymehistochemical techniques, the phenotype of the neoplastic cells in seven cases of mantle zone lymphoma (MZL) was compared to that in seven cases of nodular poorly-differentiated lymphocytic lymphoma (NPDLL). The neoplastic nodules in MZL consisted of medium-sized lymphoid cells with slightly irregular nuclei and finely dispersed chromatin, expressing monoclonal surface IgM or IgM plus IgD, and displaying membranous alkaline phosphatase (ALP) activity. These cells proliferated around follicular centers that demonstrated a polyclonal pattern of reactivity for both types of light chains and a distorted meshwork of dendritic reticulum cells. The neoplastic nodules in NPDLL consisted of small lymphoid cells with markedly irregular nuclei and coarsely granulated chromatin, expressing monoclonal surface IgM and lacking ALP-activity. These tumor cells also frequently expressed transferrin receptor and common acute lymphoblastic leukemia-antigen (CALLA). The neoplastic nodules showed an undistorted meshwork of dendritic reticulum cells, and were occasionally bordered by remnants of polyclonal lymphocytic coronas. These results confirm the previous suggestion that NPDLL arises from a cell type that is a normal constituent of follicular centers, whereas MZL arises from the lymphocytic corona. The morphological, enzyme- and immunohistochemical features of MZL cells strongly suggest that MZL arises from marginal zone lymphocytes, a subset of corona lymphocytes that expresses ALP-activity, high IgM and low IgD-levels.     

Myofibroblast differentiation: plasma membrane microdomains and cell phenotype

Myofibroblast differentiation characterizes a prominent cellular phenotype identified in experimental models of progressive kidney disease and human kidney biopsies. Mesangial cells, tubulointerstitial fibroblasts and, perhaps, tubular epithelial cells undergo myofibroblast differentiation, a process characterized by alpha-actin expression, synthesis of interstitial collagens and a growth response. Inhibition of myofibroblast differentiation could prevent kidney disease progression but may be difficult to accomplish, since inhibition of multiple signaling pathways would be required. Cell biology advances have enabled a better understanding of how information from many microenvironmental stimuli are integrated by spatial compartmentalization of extracellular receptors and cytosolic signaling molecules within specialized plasma membrane domains, such as focal adhesions and lipid rafts. We review this information and hypothesize that myofibroblast differentiation of renal cells can only proceed if the spatial arrangement of intracellular molecules, in large part determined by extracellular matrix-regulated cytoskeletal organization, permits activation of appropriate signaling pathways by soluble molecules interacting with receptors in specialized plasma membrane microdomains. If proven, this hypothesis suggests targeting key molecules within adhesion complexes and rafts (in some cases with drugs that are already clinically available) may provide more effective therapy for kidney disease progression.     

Splenic marginal zone lymphoma with or without plasmacytic differentiation

We report a series of 31 cases of splenic marginal zone lymphomas with an enlarged spleen and a multimicronodular macroscopic pattern. Two groups, A and B, were distinguished based on the presence (A) or absence (B) of a lymphoplasmacytic component with monoclonal immunoglobulin expression in the cytoplasm. There were no differences between the groups as far as age, sex, spleen weight, and progression. The only difference was the presence in group A of a monoclonal serum component and autoimmune disorders, particularly autoimmune hemolytic anemia. In most cases in which a liver and/or bone marrow biopsy was performed, lymphomatous infiltration was detected. Seven cases had a seric monoclonal IgM of 5 g/L or more and liver or bone marrow infiltration, corresponding to the definition of Waldenstrom's macroglobulinemia. Lymphoma cells had a monocytoid, centrocytoid and, in group A, lymphoplasmacytic morphology. The lymphomatous cells were positive for CD20, CD45 RA, and bcl-2. They expressed IgD in 9 cases, partially in 6, and were negative for IgD in 9 of the 24 cases studied. Progression seems to be slow, with a long survival. Three patients presented with transformation into a large B-cell lymphoma, which was responsible for death in two patients.     

Intramedullary astrocytoma with granular cell differentiation

Granular cell astrocytomas are uncommon tumors of the central nervous system (CNS) of which no cases have been documented in the spinal cord. This variant of glioma should not be confused with benign granular cell tumor which, although rare, has been well characterized in the spinal cord. We describe here the clinical, pathological, and radiological features of such an astrocytoma arising within the spinal cord at the dorsal level. A 48-year-old female was seen after about 1 year of dorsal pain and gradual spastic paraparesis. Magnetic resonance imaging (MRI) studies showed a 2-cm contrast-enhanced mass in the spinal cord at T6-T7, which had the appearance of an astrocytoma. At surgery, the tumor was found to be infiltrating a posterior column with no dural attachment. It was debulked and dissected. The histological diagnosis was astrocytoma with granular cell differentiation. In addition to documenting a unique example of intramedullary granular cell astrocytoma, we review the literature to investigate differences from other tumors with granular changes described in the spinal cord.     

Chromophobe renal cell carcinoma with osteosarcoma-like differentiation

Sarcomatoid differentiation in renal cell carcinoma is thought to be the result of the dedifferentiation of the parent tumor, and it can be found in the chromophobe renal cell carcinoma just as other subtypes. We report a case of chromophobe renal cell carcinoma, which showed osteosarcoma-like differentiation. This is the first known case ever to be clearly identified as such. The patient was a 74-year-old man, and the CT scan revealed a huge retroperitoneal mass, which protruded from the lower half of the kidney and directly invaded the colon. Intraabdominal dissemination and metastases to the liver and lungs were also found. The resected tumor histologically showed sarcoma-like spindle cell proliferation and partly produced massive osteoid, which simulated the osteosarcoma. In addition, a typical histology of chromophobe renal cell carcinoma was found in part of the tumor. Immunohistochemically, spindle cells were reactive for epithelial membrane antigen, cytokeratin, and vimentin. The cell nests that were labeled by epithelial membrane antigen and cytokeratin were also found in the osteosarcoma-like area. We think that these phenomena were the result of "dedifferentiation" and metaplasia of the chromophobe renal cell carcinoma.     

Malignant lymphoma differentiated to plasma cell of the testis: report of a case

Malignant lymphoma of the left testis was seen in a 53-year-old man. Pathologically, the tumor cell showed malignant lymphoma of the diffuse, small cell type, especially, of lympho-plasmacytoid in LSG classification. Clinically, no other lesions were found. At 26 months following orchiectomy with chemotherapy (CHOP regimen: cyclophosphamide, hydroxydaunomycine, vincristine and prednisone) as well as post-operative irradiation (60Co, 30 Gy.), the patient has been doing well without any clinical evidence of recurrence generalization of the tumor.     

Mantle cell lymphoma involving skin: cutaneous lesions may be the first manifestation of disease and tumors often have blastoid cytologic features

We describe five cases of mantle cell lymphoma involving skin. Three patients initially presented with skin lesions but had evidence of widespread disease at time of diagnosis or with relatively short follow-up. One patient was known to have disseminated disease before he developed skin lesions. One patient presented with a solitary skin nodule on the thigh and has developed multiple smaller nodules on the same leg, but no other sites of disease over 30 months of clinical follow-up. This case fulfills the criteria for primary cutaneous lymphoma as proposed by the European Organization for Research and Treatment of Cancer. Biopsy of the skin lesions in all cases showed predominantly dermal and focally subcutaneous lymphoid infiltrates, preferentially perivascular and periadnexal in four cases, and nodular in one case. The tumors were composed of small- to medium-sized lymphocytes with irregular nuclear contours. Four cases had blastoid and one case had typical cytologic features. Immunophenotypic studies showed that all cases were positive for CD20 and cyclin D1, and four of five were positive for CD5. Four cases, including the CD5-negative case, had evidence of the t(11;14) shown by either fluorescence in situ hybridization methods performed on skin tumors or conventional cytogenetic analysis performed on involved bone marrow. We conclude that mantle cell lymphoma can involve skin, usually as a manifestation of disseminated disease, and is often associated with blastoid cytologic features. Rare cases of mantle cell lymphoma may arise in skin.     

Cutaneous B cell lymphoma in a patient with leprosy

Authors report the case of a male patient treated in the Dermatology Department for cutaneous leprosy, and then sent to the Plastic Surgery Department for excisional biopsy of a giant shoulder nodule unresponsive to antibacilar chemotherapy and interpreted as a leprosy reaction. The extemporary histological result was of a cutaneous lymphoma and the defect was reconstructed with two random regional fasciocutaneous flaps, a rotation scapular flap and a transposition anterior brachial flap. The rarity of this association, not previously reported in the literature, and the success of the chosen surgical treatment motivated us to publish the case. We present the clinical evolution of the lesion, the surgical reconstructive option and the final result which we consider excellent from clinical, aesthetic and functional points of view.     

Intracranial plasma cell granuloma with genetic analysis

Summary.  Background: Plasma cell granuloma of the central nervous system is rare and less than 30 cases have been reported in literature.  Method/findings: A 64-year-old woman complained of headache, and computed tomography (CT) and magnetic resonance (MR) imaging of the brain revealed a tumour at the right anterior-third parasagittal region which led us to diagnose a parasagittal meningioma. We performed total resection of the tumour and the pathological diagnosis was plasma cell granuloma. Polymerase chain reaction (PCR) analysis of immunoglobulin heavy chain (IgH) gene rearrangements produced no monoclonal band. This patient underwent no supplementary treatment and no recurrent tumour was observed for 3 years.  Interpretation: A case of plasma cell granuloma which is diagnosed with genetic analysis is presented. An analysis of the clonality using the PCR procedure would play an important role in supporting the pathological and clinical diagnosis. Published online March 3, 2003  Correspondence: Mamoru Murakami, M.D., Department of Neurosurgery, Kyoto Prefectural University of Medicine, Kajii-cho 465, Kawaramachi Hirokoji, Kamigyo-ku, Kyoto, 602-8566, Japan.     

Glomerular endothelial cell differentiation

BACKGROUND: Glomerular endothelial cells differ from most other endothelial cells in that they are extraordinarily flattened and highly fenestrated. In this differentiated form, they allow formation of glomerular ultrafiltrate at a prodigious rate. METHODS: Molecular processes that dictate the development and differentiation of glomerular endothelium are reviewed. RESULTS: During glomerular development, angioblasts already present in the metanephric blastema well before any organized angiogenic sprouts invade the capillary cleft of developing nephrons at the comma and S-shape stages in response to chemotactic and guiding cues from primitive podocytes. The angioblasts then undergo homotypic aggregation into precapillary cords as yet devoid of a lumen. Lumen development then proceeds through the loss of superfluous endothelial cells by apoptosis as well as flattening of the remaining viable endothelial cells. The final step, fenestration, is critically dependent on appropriate stimuli, most notably vascular endothelial growth factor A (VEGF-A), from differentiated podocytes. Current evidence suggests that the fenestrae of fully differentiated glomerular endothelium can be lost within hours if the VEGF-A stimulus is removed, and that the glomerular endotheliosis, loss of glomerular filtration rate (GFR) and proteinuria observed in preeclampsia are due to the circulating inhibitor of VEGF-A, soluble VEGF receptor 1 (VEGFR-1). CONCLUSION: Differentiation of the glomerular endothelium is highly dependent on podocyte-derived stimuli and their loss leads to the derangements of glomerular function in preeclampsia.     

Primary cardiac T cell lymphoma

We report a case of primary cardiac T-cell lymphoma. The diagnosis and management of this unusual primary tumor is reviewed.     

Anaplastic large cell lymphoma.

Anaplastic large cell lymphoma (ALCL) or Ki-1 lymphoma is a recently described and distinctive non-Hodgkin's lymphoma. Cervicofacial adenopathy caused by ALCL may mimic involvement by metastatic carcinoma or other malignancies common to the head and neck. A case in which ALCL was originally interpreted as metastatic nasopharyngeal carcinoma is presented. The clinicopathologic features of this uncommon entity are discussed.     

Epidural B cell non-Hodgkin's lymphoma associated with chronic subdural hematoma

BACKGROUND: Chronic subdural hematoma generally occurs in elderly patients, who are also prone to neoplasm. However, the association of chronic subdural hematoma and intracranial neoplasm is rare. CASE DESCRIPTION: An elderly woman harboring a chronic subdural hematoma underlying an epidural extension of a malignant B cell non-Hodgkin's lymphoma is reported. Infiltration of the tumor and obstruction of the dural veins with stasis and rupture of the capillary net may have been the mechanism that led to formation of the hematoma. CONCLUSIONS: In elderly patients harboring chronic subdural hematoma with mural isodensity on CT who have no history or an uncertain history of head trauma, one should consider the possibility of neoplasm, although it is rare. Chronic subdural hematoma associated with epidural extension of B cell non-Hodgkin's lymphoma is exceptional. Screening for other systemic manifestations should be carried out. Radiotherapy, alone or in combination with chemotherapy, should be considered after surgical removal.