Steroidogenesis in ovarian-like mesenchymal stroma of hepatic and pancreatic mucinous cystic neoplasms.

STEROIDOGENESIS IN HEPATIC MUCINOUS CYSTIC NEOPLASM: Aim Mucinous cystic neoplasms (MCNs) occur in the ovary, pancreas, and retroperitoneum but very rarely in the liver. Mucinous cystic neoplasms are known to harbor ovarian-like mesenchymal stroma (OLS) expressing progesterone and estrogen receptors. In this study we evaluated steroidogenesis in OLS of 25 hepatic MCNs and 24 pancreatic MCNs. Methods Both steroid receptors and steroidogenic factors were immunohistochemically evaluated using H-scores and results were compared with those in 15 ovarian MCNs and 10 normal ovaries. Results Androgen receptor (AR) H-scores in OLS were significantly higher in hepatic, pancreatic, and ovarian MCN than those in normal ovaries. H-scores of cytochrome P450 17α-hydroxylase/c17-20 lyase (P450c17) and 5α-reductase-1 (5αRED-1) in the stroma were significantly higher in OLS of hepatic and pancreatic MCN than in the stroma of ovarian MCN and normal ovary. In tumor epithelium, AR H-scores were significantly higher in hepatic and pancreatic MCN than in ovarian MCN. In both hepatic and pancreatic MCN, a significant positive correlation was detected between AR H-score in the epithelium and P450c17 H-score in OLS (hepatic MCN: Pearson's r = 0.446, P = 0.025; pancreatic MCN: r = 0.432, P = 0.035). In pancreatic MCN, a significantly positive correlation was detected between AR H-score in the tumor epithelium and 5αRED-1 H-score in OLS (Pearson's r = 0.458, P = 0.024). Conclusions These results indicated that locally produced androgens in OLS could be pivotal for tumorigenesis of both hepatic and pancreatic MCN and influence epithelial cells, possibly in a paracrine fashion, which could represent biological significance of OLS in these neoplasms.

[A case of transverse-sigmoid sinus dural arteriovenous fistula that became symptomatic after removal of coexisting sphenoid ridge meningioma].

Here, we describe a case with a transverse-sigmoid sinus(TSS)dural arteriovenous fistula(DAVF)with sinus occlusion at its proximal and distal ends;the TSS DAVF turned symptomatic after removal of a coexisting symptomatic sphenoid ridge meningioma. The patient was a 70-year-old man presenting with decreased daily activities and mentation due to dysphasia. Magnetic resonance imaging revealed a large, left sphenoid ridge meningioma. Angiography revealed a tumor stain and a coexisting left TSS DAVF with sinus occlusion at its proximal and distal ends. Cortical venous reflux(CVR)into the temporal veins was observed. After successful tumor removal, the superficial middle cerebral vein was arterialized intraoperatively. However, the patient showed worsened consciousness and dysphasia after the operation. Repeated angiography revealed CVR into the superficial middle cerebral vein through a sphenopetrosal sinus. Transvenous embolization was performed via the contralateral inferior petrosal and intercavernous sinus, which allowed access to the ipsilateral superior petrosal sinus(SPS);the procedure successfully eliminated CVR, while preserving the SPS. The patient demonstrated full recovery. This case exemplifies a coexisting TSS DAVF after tumor resection, and superficial middle cerebral vein decompression due to the sphenopetrosal sinus, an alternate drainage pathway for the superficial middle cerebral vein.

Diffuse large B-cell lymphoma initially manifesting in the bone marrow.

We histologically and immunohistochemically studied 37 cases of diffuse large B-cell lymphoma (DLBCL) initially manifesting in the bone marrow (BM). We also compared these cases with the Asian variant of intravascular large B-cell lymphoma (AIVL). Histologically, the neoplastic cells of the BM mostly had large and round nuclei and formed clusters. Immunohistochemically, all cases were positive for B-cell markers. Factor VIII staining revealed neoplastic cells within the sinusoids of BM in 8 cases; however, these cells accounted for fewer than 20% of the overall neoplastic cells. In several cases, the neoplastic cells infiltrated liver, spleen, kidneys, lungs, stomach, and adrenal glands with a mainly leukemic and infrequently intravascular pattern. Although our cases share some clinical features with AIVL, we consider DLBCL initially manifesting in the BM to be a unique entity because the neoplastic cells proliferate mainly in BM, with infrequent involvement of the sinusoids and occasional leukemic infiltration in various organs.

XCR1 expression and biased VH gene usage are distinct features of diffuse large B-cell lymphoma initially manifesting in the bone marrow.

A total of 29 cases of diffuse large B-cell lymphoma initially manifesting in the bone marrow (BM-DLBCL) were analyzed for V(H) gene sequence, and expression microarray of chemokines and chemokine receptors and immunohistochemical analysis were done. Seminested polymerase chain reaction (PCR) and sequencing analyses of 18 cases revealed that the V(H) gene usage in 6 cases was restricted to V(H)3-7, in 3 cases to V(H)4-34, and in 2 cases to V(H)4-39, which were all previously reported to be autoreactive. In total, 14 of 18 V(H) genes were those associated with autoimmune diseases, including V(H)3-21, V(H)3-23, and V(H)3-48. Furthermore, cDNA microarray analysis specific for chemokine and chemokine receptors revealed that chemokine receptor XCR1 expression was significantly elevated in the BM-DLBCL cases (P < .05), which was confirmed by quantitative reverse transcriptase-PCR and immunohistochemical analysis. Expression of the chemokine receptor XCR1 and frequent usage of autoreactive V(H) genes seem to be distinct characteristics of BM-DLBCL.