Expression of FK506-binding protein 51 (FKBP51) in Mycosis fungoides.

BACKGROUND: Mycosis fungoides (MF) is the major subtype of cutaneous T-cell lymphomas (CTCL). It usually has a prolonged indolent clinical course with a minority of cases acquiring a more aggressive biological profile and resistance to conventional therapies, partially attributed to the persistent activation of nuclear factor-kappa B (NF-κB) pathway. In the last decade, several papers suggested an important role for the FK506-binding protein 51 (FKBP51), an immunophilin initially cloned in lymphocytes, in the control of NF-κB pathway in different types of human malignancies. OBJECTIVES: We aimed to investigate the possible value of FKBP51 expression as a new reliable marker of outcome in patients with MF. METHODS: We assessed by immunohistochemistry (IHC) FKBP51 expression in 44 patients with MF, representative of different stages of the disease. Immunohistochemical results were subsequently confirmed at mRNA level with quantitative PCR (qPCR) in a subset of enrolled patients. In addition, IHC and qPCR served to study the expression of some NF-κB-target genes, including the tumour necrosis factor receptor-associated factor 2 (TRAF2). RESULTS: Our results show that FKBP51 was expressed in all evaluated cases, with the highest level of expression characterizing MFs with the worst prognosis. Moreover, a significant correlation subsisted between FKBP51 and TRAF2 IHC expression scores. CONCLUSIONS: We hypothesize a role for FKBP51 as a prognostic marker for MF and suggest an involvement of this immunophilin in deregulated NF-κB pathway of this CTCL.

Randomized comparison of power Doppler ultrasonography-guided core-needle biopsy with open surgical biopsy for the characterization of lymphadenopathies in patients with suspected lymphoma.

The sensitivity of lymph node core-needle biopsy under imaging guidance requires validation. We employed power Doppler ultrasonography (PDUS) to select the lymph node most suspected of malignancy and to histologically characterize it through the use of large cutting needle. Institutional review board approval and informed consent were obtained for this randomized clinical trial. In a single center between 1 January 2009 and 31 December 2015, patients with lymph node enlargement suspected for lymphoma were randomly assigned (1:1) to biopsy with either standard surgery or PDUS-guided 16-gauge modified Menghini needle. The primary endpoint was the superiority of sensitivity for the diagnosis of malignancy for core-needle cutting biopsy (CNCB). Secondary endpoints were times to biopsy, complications, and costs. A total of 376 patients were randomized into the two arms and received allocated biopsy. However, four patients undergoing CNCB were excluded for inadequate samples; thus, 372 patients were analyzed. Sensitivity for the detection of malignancy was significantly better for PDUS-guided CNCB [98.8%; 95% confidence interval (CI), 95.9-99.9] than standard biopsy (88.7%; 95% CI, 82.9-93; P < 0.001). For all secondary endpoints, the comparison was significantly disadvantageous for conventional approach. In particular, estimated cost per biopsy performed with standard surgery was 24-fold higher compared with that performed with CNCB. The presence of satellite enlarged reactive and/or necrotic lymph nodes may impair the success of an open surgical biopsy (OSB). PDUS and CNCB with adequate gauge are diagnostic tools that enable effective, safe, fast, and low-cost routine biopsy for patients with suspected lymphoma, avoiding psychological and physical pain of an unnecessary surgical intervention.

Protein-loosing enteropathy in sclerosing mesenteritis.

Sclerosing mesenteritis (SM) is a rare, idiopathic disorder of unknown aetiology that involves the adipose tissue of the mesentery, being characterized by chronic and non-specific fibrous inflammation. Patients usually present with non-specific clinical manifestations, such as abdominal pain and diarrhoea. The diagnosis of SM is difficult and it can be definitely established only by means of surgical or imaging-guided biopsy. Different therapeutic strategies have been used in case series with different rate of success. The disease is generally self-limiting, and the long-term prognosis is good, even if some cases of severe SM are reported in literature. Here, we report a fatal case of sclerosing mesenteritis associated to protein-losing enteropathy.

A case of extraovarian primary peritoneal carcinoma in an oophorectomized-hysterectomized patient: a diagnostic dilemma.

Extra Ovarian Primary Peritoneal Carcinoma (EOPPC) is a rare type of adenocarcinoma of the pelvic and abdominal peritoneum. The objective examination and the histological aspect of the neoplasia virtually overlaps with that of ovarian carcinoma. The reported case is that of a 72 year-old patient who had undergone a total hysterectomy with bilateral annessiectomy surgery 20 years earlier subsequently to a diagnosis for uterine leiomyomatosis. The patient came to our attention presenting recurring abdominal pain, constipation, weight loss, severe asthenia and fever. Her blood test results showed hypochromic microcytic anemia and a remarkable increase CA125 marker levels. Instrumental diagnostics with Ultrasound (US) and CT scans indicated the presence of a single peritoneal mass (10-12 cm diameter) close to the great epiploon. The patient was operated through a midline abdominal incision and the mass was removed with the great omentum. No primary tumor was found anywhere else in the abdomen and in the pelvis. The operation lasted approximately 50 minutes. The post-operative course was normal and the patient was discharged four days later. The histological exam of the neoplasia, supported by immunohistochemical analysis, showed a significant positivity for CA 125, vimentin and cytocheratin, presence of psammoma bodies, and cytoarchitectural pattern resembling that of a serous ovarian carcinoma even in absence of primitiveness, leading to a final diagnosis of EOPPC. The patient later underwent six cycles of chemotherapy with paclitaxel (135 mg/m²/24 hr) in association with cisplatin (75mg/m²). At the fourth year follow-up no sign of relapse was observed.

Large idiopathic unilateral adrenal hematoma in a young woman.

This is a case report on a young woman with a large idiopathic unilateral adrenal hematoma (AH). Only few cases of AH which were not associated with any trauma, previous surgery, coagulative or any other systemic disorders have been described. The mass was discovered by abdominal ultrasound which was performed for a recent flank pain. Magnetic resonance imaging (MRI) confirmed the presence of a 13-cm sized lesion in the right hemi-abdomen; T1 and T2 weighed imaging was compatible with subacute-to-chronic adrenal hematoma. The lesion dislocated the liver and right kidney. Positron emission tomography (PET) did not show any significant radiotracer uptake by the mass. Serum cortisol, aldosterone, renin activity and DHEA-S were normal. Urinary catecholamines and free cortisol excretion were within the normal range too. The lesion was removed by transabdominal laparoscopic adrenalectomy without any complication. The histological exam confirmed a large subacute- to-chronic organized AH. In conclusion, in the absence of known risk factors, differential diagnosis of a large AH may not be easy. The possibility of an underlying pheochromocytoma, malignant adrenal or metastatic tumor must always be considered. In our patient, computed tomography (CT) scan and MRI suggested the presence of a large subacute-to-chronic AH, and PET excluded metabolic activity of the mass. Laparoscopic adrenalectomy can be the surgical treatment of choice in organized symptomatic AH. The correct diagnosis, early recognition and treatment of complications including adrenal insufficiency may decrease patient morbidity and mortality.

Intra-abdominal extra lobar pulmonary sequestration communicating with gastric duplication -- a case report.

Gastric duplications with associated pulmonary sequestration are very infrequent abnormalities. Although often asymptomatic, they should be considered in the differential diagnosis of intra-abdominal or retro-peritoneal masses. The authors report the case of a ten-year-old boy who had an occasional finding of poorly symptomatic intra-abdominal mass, recognised at intervention as a gastric duplication with associated extralobar pulmonary sequestration. The authors describe the surgical treatment of this case and briefly discuss the current views on the embryological pathogenesis of such malformations.