Solitary Cranial Langerhans Cell Histiocytosis : Two case reports

Langerhans cell histiocytosis (LCH) is a proliferation of Langerhans cells intermixed with inflammatory cells, in particular eosinophils, that may manifest as a unisystem (unifocal or multifocal) or multisystem disease. We describe the clinical and histologic spectrum of LCH of the orbit and skull in our two cases. Both cases had unifocal erosive skull lesions with a history of trauma. Typical histologic features included numerous histiocytes with varying degrees of giant cell formation and scattered eosinophilic granulocytes. The presence of Langerhans cells was confirmed by CD1a and S100 immunohistochemistry. LCH has an excellent prognosis when treated with surgical resection, steroids and radiotherapy or chemotherapy. One of our patients is disease free at 7 year follow-up and one patient had regression of lesion on follow-up

Robotic Renal Autotransplantation: First Case Outside of North America

A 38-year-old woman with a 2.7-cm left ureteral stenosis requiring chronic ureteral stent exchange elected to undergo robotic renal autotransplantation. Left ureteropelvic junction obstruction (UPJO) was also suspected. Robotic donor nephrectomy contributed to the fine dissection for desmoplastic changes. The kidney was removed through a Gelport and examined on ice. UPJO was not seen. An end-to-side robotic anastomosis was created between the renal and external iliac vessels. The console time was 507 min, and the warm ischemia time was 4 min 5 sec. She became stent-free. Robotic renal autotransplantation is a new, minimally invasive approach to renal preservation

Clinicopathologic Features in a TSH Secreting Pituitary Tumor : a Case Report

Thyrotropin-stimulating hormone-producing adenoma (TSHoma) is a rare cause of hyperthyroidism. Patients with this condition are often diagnosed when the tumor has already grown significantly, as the diagnosis of TSHoma is frequently delayed and confused with primary hyperthyroidism. Our case describes a 60-year-old woman with a goiter who was referred to our center from the otolaryngology department. Her thyroid panel in endocrinology showed elevated free hormone levels (fT3: 4.6 mg/ml and fT4: 1.93 mg/ml) and an unsuppressed TSH: 12.85 μ mIU/l, suggesting central hyperthyroidism. A thyroid gland sample obtained by a fine needle aspiration biopsy did not show malignant cells. An MRI scan of the pituitary gland revealed a macroadenoma with KNOSP grade 1. She underwent transsphenoidal surgery for the tumor. Histopathology revealed an acidophilic adenoma with the expression of TSH and prolactin by immunostaining. The proliferation rate, as indicated by MIB-1 staining, was only 0.6%. The decrease in thyroid hormones after the operation confirmed the effectiveness of the surgery. In conclusion, TSHoma, a rare tumor associated with hyperthyroidism, requires a careful diagnosis for effective management. Our article serves as reference material for understanding cases of pituitary-secreting tumors in Indonesia

Rathke’s cleft-like cysts arise from Isl1 deletion in murine pituitary progenitors

The transcription factor ISL1 is expressed in pituitary gland stem cells and the thyrotrope and gonadotrope lineages. Pituitary-specific Isl1 deletion causes hypopituitarism with increased stem cell apoptosis, reduced differentiation of thyrotropes and gonadotropes, and reduced body size. Conditional Isl1 deletion causes development of multiple Rathke’s cleft-like cysts, with 100% penetrance. Foxa1 and Foxj1 are abnormally expressed in the pituitary gland and associated with a ciliogenic gene-expression program in the cysts. We confirmed expression of FOXA1, FOXJ1, and stem cell markers in human Rathke’s cleft cyst tissue, but not craniopharyngiomas, which suggests these transcription factors are useful, pathological markers for diagnosis of Rathke’s cleft cysts. These studies support a model whereby expression of ISL1 in pituitary progenitors drives differentiation into thyrotropes and gonadotropes and without it, activation of FOXA1 and FOXJ1 permits development of an oral epithelial cell fate with mucinous cysts. This pituitary-specific Isl1 mouse knockout sheds light on the etiology of Rathke’s cleft cysts and the role of ISL1 in normal pituitary development.Fil: Brinkmeier, Michelle L.. University of Michigan; Estados UnidosFil: Bando, Hironori. University of Michigan; Estados UnidosFil: Camarano, Adriana Carla. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Ciudad Universitaria. Instituto de Fisiología, Biología Molecular y Neurociencias. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Instituto de Fisiología, Biología Molecular y Neurociencias; ArgentinaFil: Fujio, Shingo. Kagoshima University; JapónFil: Yoshimoto, Koji. Kagoshima University; JapónFil: Silva Junqueira de Souza, Flávio. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Ciudad Universitaria. Instituto de Fisiología, Biología Molecular y Neurociencias. Universidad de Buenos Aires. Facultad de Ciencias Exactas y Naturales. Instituto de Fisiología, Biología Molecular y Neurociencias; ArgentinaFil: Camper, Sally. University of Michigan; Estados Unido