Ectopic sphenoidal ACTH-secreting adenoma revealed by 11C Methionine PET scan: case report.

BACKGROUND: Ectopic ACTH pituitary adenomas (EAPA), located outside the sella turcica and deriving from cellular remnants of Rathke's pouch are a very rare cause of Cushing's syndrome (CS). The diagnosis is often difficult and delayed, even after comprehensive work-up. To our knowledge, we report for the first time an ectopic corticotroph tumor of the posterior wall of the sphenoid sinus, leading to false positive results of bilateral inferior petrosal sinus sampling (BIPPS) and which was finally localized by a co-registered11 C Methionine PET/MR imaging. CASE PRESENTATION: A 48-year-old woman was referred for a high clinical suspicion of ACTH-dependent CS. Biological testing comprising low dose dexamethasone suppression and CRH stimulation tests were indicative of pituitary Cushing's disease, but comprehensive pituitary MRI did not reveal any pituitary adenoma. BIPSS confirmed however a central origin of ACTH secretion (central-to-peripheral ACTH ratio > 100) and revealed a significant right-to-left gradient (6.2), leading to a first right-sided exploratory hypophysectomy, that did not cure the patient. BIPSS images were reviewed and revealed preferential drainage of the left pituitary to the right petrosal sinus, leading us to a left sided exploratory hypophysectomy, which was again unsuccessful. A11 C Methionine PET/MRI was performed and revealed a hypermetabolic lesion adjacent to the posterior wall of the sphenoidal sinus. After surgical resection, this polypoid mass was identified as an ectopic ATCH-secreting pituitary adenoma expressing ACTH and T-Pit and complete remission of hypercortisolism was observed. CONCLUSIONS: In conclusion, we report a case of ACTH-dependent Cushing's syndrome, caused by an ectopic corticotroph adenoma located in the sphenoidal sinus, which perfectly mimicked the biological features of a classical pituitary ACTH adenoma on a comprehensive hormonal evaluation including BIPPS, and the features of a benign naso-sinusal polyp at MRI. We report for the first time a key role of11 C Methionine PET co-registered to high resolution MRI for localizing ectopic adenomas, efficiently guiding surgical removal and leading to complete remission of hypercortisolism.

Combination of a six microRNA expression profile with four clinicopathological factors for response prediction of systemic treatment in patients with advanced colorectal cancer.

BACKGROUND: First line chemotherapy is effective in 75 to 80% of patients with metastatic colorectal cancer (mCRC). We studied whether microRNA (miR) expression profiles can predict treatment outcome for first line fluoropyrimidine containing systemic therapy in patients with mCRC. METHODS: MiR expression levels were determined by next generation sequencing from snap frozen tumor samples of 88 patients with mCRC. Predictive miRs were selected with penalized logistic regression and posterior forward selection. The prediction co-efficients of the miRs were re-estimated and validated by real-time quantitative PCR in an independent cohort of 81 patients with mCRC. RESULTS: Expression levels of miR-17-5p, miR-20a-5p, miR-30a-5p, miR-92a-3p, miR-92b-3p and miR-98-5p in combination with age, tumor differentiation, adjuvant therapy and type of systemic treatment, were predictive for clinical benefit in the training cohort with an AUC of 0.78. In the validation cohort the addition of the six miR signature to the four clinicopathological factors demonstrated a significant increased AUC for predicting treatment response versus those with stable disease (SD) from 0.79 to 0.90. The increase for predicting treatment response versus progressive disease (PD) and for patients with SD versus those with PD was not significant. in the validation cohort. MiR-17-5p, miR-20a-5p and miR-92a-3p were significantly upregulated in patients with treatment response in both the training and validation cohorts. CONCLUSION: A six miR expression signature was identified that predicted treatment response to fluoropyrimidine containing first line systemic treatment in patients with mCRC when combined with four clinicopathological factors. Independent validation demonstrated added predictive value of this miR-signature for predicting treatment response versus SD. However, added predicted value for separating patients with PD could not be validated. The clinical relevance of the identified miRs for predicting treatment response has to be further explored.

[Late-occurring cutaneous vasculitis after successful treatment of diffuse lepromatous leprosy: Lucio's phenomenon]. / Vasculite cutanée a révélation tardive après guérison d'une lèpre lépromateuse diffuse: le phénomène de Lucio.

O Lucio's phenomenon is an uncommon type 2 reactional state occurring exclusively in patients with diffuse lepromatous leprosy (Lucio-Latapi leprosy). Previous case reports have been most frequent in Central America and rare in Asia and Africa. Lucio's phenomenon is characterized by necrotic ulcerations of the skin preferentially on the lower extremities usually in association with ongoing Lucio lepromatosis. The purpose of this report is to describe an unusual case of Lucio's phenomenon occurring four years after successful treatment of diffuse lepromatous leprosy. The patient was a 51-year-old man who had presented diffuse lepromatous leprosy ongoing since 1998. Diagnosis was documented based on histological and bacteriologic evidence. After successful treatment using dapsone (100 mg/d), rifadine (600 mg/month) and ethionamide (250 mg/d), the patient was lost from follow-up for 4 years. In January 2005, he consulted again for alteration of general status. Clinical examination showed inflammatory livedo on the lower extremities in association with several infiltrating maculo-papular lesions and painful erythemato-pupuric lesions on the legs and buttocks. The patient's skin was dry, shiny and galabrous with alopecia of the eyelashes and eyebrows. Examination of smear samples (skin and nasal) to identify mycobacterium leprae was negative. Histological study demonstrated epidermic necrosis with aspects of leucocytoclastic vasculitis. No Virchow cells were detected and Ziehl staining was negative. Search for circulating immune complexes and antiphospholipid antibodies was negative. Diagnosis of Lucio's phenomenon was made and the patient was treated using prednisone at a dose of 1 mg/kg/d in association with rifampicine (600 mg/month) and dapsone (100 mg/d). Outcome was favorable after one month of treatment. Lucio's phenomenon has rarely been observed in Tunisia. To our knowledge this is the third case reported from Tunisia and only 13 cases have been reported in the world since 1983. In all cases including the two from Tunisia, Lucio's phenomenon occurred during the course of treatment of ongoing Lucio-Latapi lepromatous leprosy (2). The remarkable features of our case are that Lucio's phenomenon occurred a long time after successful treatment of lepromatous leprosy and that the patient responded promptly to treatment. The pathogenesis of Lucio's phenomenon is often compared with that of erythema nodosum leprosum. Discussion focuses on pathophysiologic features and natural course of Lucio's phenomenon.

[Management of severe psoriasis: about 50 cases]. / Modalites therapeutiques dans les formes severes de psoriasis: a propos de 50 cas.

BACKGROUND: With its varied clinical presentation, psoriasis is actually considered as a public health care problem. Severe psoriasis remains difficult to treat. METHODS: Our study was retrospective involving 50 patient with severe psoriasis treated in the dermatology unit of military hospital of Tunis from January 1990 to December 2003. Aim of our study was to evaluate actual different therapeutic attitudes and new treatments in case of severe psoriasis. RESULTS: During the 14 years studied the frequency of severe psoriasis was about 19.5%. Extended psoriasis was noticed on 64% of the patients. Arthropatic psoriasis was found on only 6% of the patients. Erythrodermic psoriasis, pustular psoriasis and palmoplantar psoriatic keratoderma were equally noticed in 10% of the patients. PUVA-therapy was firstly prescribed on 96.6% of the patients with extended psoriasis, bettering was noticed on 71% of all them during the 3 first cures. From the 20 patients that have received acitretin, 65% have noticed improvement. Méthotrexate and Re-PUVA was secondarily used (only for the cases of psoriasic arthritis amd méthotrexate) on respectively 18% and 10% of the patients. All our patients reported worsening on thems quality of life. CONCLUSION: Cyclosporin is more and more used initially in cases of rebellious psoriasis. Actually therapeutic hope leans on biological therapies.

[Erythema nodosum: profile in a Tunisian teaching hospital]. / Erythemes noueux: etude d'une serie hospitaliere.

BACKGROUND: Erythema nodosum (EN) is the most frequent acute nodular hypodermatitis. Etiologies of EN are varied although a similar clinical presentation. The aim of our study is to establish epidemio-clinical characteristics of EN in 103 patients. METHODS: (62 in care (january 1980-december 2005) and 41 consultants (january 1991-december 2005) were seen on the dermatology unit of the Rabta hospital. We have only included patients that had done: blood count, chest X-Ray, ASLO, tuberculin intradermoreaction, transaminase, inflammation marker. RESULTS: We had 86 women and 17 men with a mean age of 36,5 years. Lesions were located in lower limbs in 88 cases. The main etiologies were: post streptococcal infections (42 cases), tuberculosis (8 cases), Crohn disease (8 cases), Behçet disease (7 cases) and sarcoidosis (5 cases). EN was idiopathic in 23 patients. The most common provoking agent of EN varies from a country to another and even in the same country from a study to another. In Tunisia, post streptococcal infections and Behçet disease still frequent etiologies for EN, sarcoidosis and inflammatory colites are more and more found unlike tuberculosis which is less incriminated than before. In case of EN it is reasonable to begin etiological enquiry by common causes.

Subcutaneous fat necrosis: report of two cases.

Subcutaneous fat necrosis of the newborn is an uncommon, benign process in full-term infants or postmature neonates who experienced a perinatal distress. It is a transient condition of unknown origin; however, hypercalcemia may be a potentially life-threatening complication of this otherwise self-healing process. We report two cases of subcutaneous fat necrosis and discuss the clinical features, etiology, and complications of the disease.

[Preparation and testing of Sardinella peptones: application to lipase production by Staphylococcus sp]. / Elaboration de peptones de Sardinelle: application à la production de lipase par la souche Staphylococcus sp.

Production of lipase by Staphylococcus sp. in media containing fish peptones from sardinelle (Sardinella aurita) prepared in the laboratory was studied. Lipase production is strongly affected by lipids present in fish flours. Fish peptones prepared from dIgresed whole flesh was an excellent substrate for lipase production. A comparison of lipase production in media containing fish peptones or high quality commercial peptones indicated that fish peptones enhanced enzyme formation.