Endodermal sinus tumor of the paranasal sinuses

BACKGROUND: We report a rare case of endodermal sinus tumor (EST) of the paranasal sinus in a 59-year-old man with a 6-week history of nasal obstruction. METHODS AND RESULTS: High-resolution MRI showed a right nasoethmoid mass with obstructive changes in the sphenoid, frontal, and right maxillary sinuses and orbital and intracranial extension. Histologic analysis showed 2 distinct histologic features: poorly differentiated carcinoma and EST. Immunohistochemical studies showed strong keratin staining in both components and restricted positivity of alpha-fetoprotein in the endodermal sinus-like component. The patient underwent 3 cycles of neoadjuvant chemotherapy consisting of ifosfamide, paclitaxel, and cisplatin, which resulted in significant regression of the tumor, but after the fourth cycle, the mass showed a slight increase in size. The tumor was excised with clear margins through an anterior craniofacial approach to the skull base. Histologic examination of the resected specimen showed extensive fibrosis with residual areas of viable tumor composed mainly of the poorly differentiated component with only residual microscopic foci of EST. Adjuvant postoperative intensity-modulated radiation therapy was administered. At 1-year follow-up, the patient was tumor free, with normal alpha-fetoprotein levels. CONCLUSION: Because of the rarity of this entity, no standardized treatment protocol has been defined. The involvement of the anterior skull base in our case necessitated a radical craniofacial resection, despite a partial response to chemotherapy.     

Congenital spinal tuberculosis associated with asymptomatic endometrial tuberculosis: A rare case report

Spinal tuberculosis constitutes 50% of all musculoskeletal tuberculosis. However, literature regarding congenital spinal tuberculosis is very scanty. Congenital spinal tuberculosis was diagnosed in a two-month-old child on the basis of age at presentation (gibbus since three weeks of age), hepatomegaly, raised ESR, radiological destruction of D10-D11 vertebrae, asymptomatic maternal endometrial tuberculosis and tuberculous histopathology from CT guided biopsy specimen from D10 vertebra. Both child and mother were treated by antitubercular treatment. Child improved symptomatically, gibbus became less prominent and ESR became normal at the end of one year of treatment. Patient had no recurrence during last two years of follow-up. This is the first case of congenital tuberculosis of spine with a documented source of infection from asymptomatic maternal endometrial tuberculosis. CT guided core biopsy from vertebra/aspiration from the paravertebral abscess help in early diagnosis and treatment to reduce neurological morbidity and mortality. Endometrial biopsy help in establishing the diagnosis of congenital tuberculosis and adequate antitubercular treatment in such cases may provide protection to fetus in subsequent pregnancy.     

Desmoplastic fibroma: a report of three cases at unusual locations

Desmoplastic fibroma of bone is an extremely rare locally aggressive primary bone tumour. Diagnosis is tricky and treatment experience limited with tumor surgeons. We studied three rare cases of desmoplastic fibroma arising primarily in bone at unusual sites. Diagnosis was established by radio-histopathologic correlation. Patients were operated with various modalities. Results were evaluated over long term follow-up (6-9 years). The disease was eradicated in all the three cases and no recurrence was seen over long term follow-up. Functional outcome was good and no complications were encountered. Wide resection of the tumor is favoured as the recurrence rates after surgical treatment are high.     

IFN-gamma-induced expression of MUC4 in pancreatic cancer cells is mediated by STAT-1 upregulation: a novel mechanism for IFN-gamma response

MUC4 is a transmembrane mucin, which is aberrantly expressed in pancreatic adenocarcinoma with no detectable expression in the normal pancreas. Here, we present a novel mechanism of IFN-gamma-induced expression of MUC4 in pancreatic cancer cells. Our studies highlight the upregulation of STAT-1 as a basis for MUC4 induction and demonstrate that its activation and upregulation by IFN-gamma are two distinct, albeit temporally integrated, signalling events that drive the selective induction of IRF-1 and MUC4, respectively, within a single cell system. The profile of interferon regulatory factor (IRF)-1 gene induction by IFN-gamma is consistent with its rapid transactivation by phospho-Y701-STAT-1. In contrast, the induction of the MUC4 mucin gene expression is relatively delayed, and occurs only in response to an increase in STAT-1 expression. A progressive binding of STAT-1 to various gamma-interferon-activated sequences (GAS) in the MUC4 promoter is observed in chromatin immunoprecipitation assay, indicating its direct association. Stimulation of STAT-1 expression by double-stranded polynucleotides or ectopic expression is shown to induce MUC4 expression, without Y701 phosphorylation of STAT-1. This effect is abrogated by short interfering RNA (siRNA)-mediated inhibition of STAT-1 expression, supporting further the relevance of STAT-1 in MUC4 regulation. In conclusion, our findings identify a novel mechanism for MUC4 regulation in pancreatic cancer cells and unfold new perspectives on the foundation of IFN-gamma-dependent gene regulation.     

Accuracy of electrocardiographic criteria for atrial enlargement: validation with cardiovascular magnetic resonance

Purpose

In mitral valve prolapse, determining whether the valve is suitable for surgical repair depends on the location and mechanism of regurgitation. We assessed whether cardiovascular magnetic resonance (CMR) could accurately identify prolapsing or flail mitral valve leaflets and regurgitant jet direction in patients with known moderate or severe mitral regurgitation.

Methods

CMR of the mitral valve was compared with trans-thoracic echocardiography (TTE) in 27 patients with chronic moderate to severe mitral regurgitation due to mitral valve prolapse. Contiguous long-axis high temporal resolution CMR cines perpendicular to the valve commissures were obtained across the mitral valve from the medial to lateral annulus. This technique allowed systematic valve inspection and mapping of leaflet prolapse using a 6 segment model. CMR mapping was compared with trans-oesophageal echocardiography (TOE) or surgical inspection in 10 patients.

Results

CMR and TTE agreed on the presence/absence of leaflet abnormality in 53 of 54 (98%) leaflets. Prolapse or flail was seen in 36 of 54 mitral valve leaflets examined on TTE. CMR and TTE agreed on the discrimination of prolapse from flail in 33 of 36 (92%) leaflets and on the predominant regurgitant jet direction in 26 of the 27 (96%) patients. In the 10 patients with TOE or surgical operative findings available, CMR correctly classified presence/absence of segmental abnormality in 49 of 60 (82%) leaflet segments.

Conclusion

Systematic mitral valve assessment using a simple protocol is feasible and could easily be incorporated into CMR studies in patients with mitral regurgitation due to mitral valve prolapse.     

Laparoscopic heminephrectomy in L-shaped crossed fused ectopia

L-shaped fused ectopic kidney is a rare variant of crossed fused renal ectopia. We describe a case of crossed fused L-shaped kidney with congenital ureteropelvic junction obstruction involving an orthotopically located kidney presenting as renal trauma, and its management by laparoscopic heminephrectomy.     

Human MUC4 mucin induces ultra-structural changes and tumorigenicity in pancreatic cancer cells

MUC4 is a type-1 transmembrane glycoprotein and is overexpressed in many carcinomas. It is a heterodimeric protein of 930 kDa, composed of a mucin-type subunit, MUC4alpha, and a membrane-bound growth factor-like subunit, MUC4beta. MUC4 mRNA contains unique 5' and 3' coding sequences along with a large variable number of tandem repeat (VNTR) domain of 7-19 kb. A direct association of MUC4 overexpression has been established with the degree of invasiveness and poor prognosis of pancreatic cancer. To understand the precise role of MUC4 in pancreatic cancer, we engineered a MUC4 complementary DNA construct, mini-MUC4, whose deduced protein (320 kDa) is comparable with that of wild-type MUC4 (930 kDa) but represents only 10% of VNTR. Stable ectopic expression of mini-MUC4 in two human pancreatic cancer cell lines, Panc1 and MiaPaCa, showed that MUC4 minigene expression follows a biosynthesis and localisation pattern similar to the wild-type MUC4. Expression of MUC4 resulted in increased growth, motility, and invasiveness of the pancreatic cancer cells in vitro. Ultra-structural examination of MUC4-transfected cells showed the presence of increased number and size of mitochondria. The MUC4-expressing cells also demonstrated an enhanced tumorigenicity in an orthotopic xenograft nude mice model, further supporting a direct role of MUC4 in inducing the cancer properties. In conclusion, our results suggest that MUC4 promotes tumorigenicity and is directly involved in growth and survival of the cancer cells.