Giant supra and retrosellar glioependymal cyst presenting with only precocious puberty. Clinical study and review of the literature.

INTRODUCTION AND IMPORTANCE: Intracranial glioependymal cysts are an uncommon type of neuroepithelial cyst and are encountered much less frequently than arachnoid cysts. These cysts primarily manifest within the parenchyma of the brain, although exceedingly rare instances have been reported in the lateral ventricles. CASE PRESENTATION: We present a highly unusual case of a glioependymal cyst in a 7-year-old girl. The glioependymal cyst was located in the midline in the suprasellar region and extended to the upper clivus region. Its only manifestation was precocious puberty. We performed endoscopic fenestration of the cyst, leading to a return of hormonal levels to normal and a slight reduction in cyst size. CLINICAL DISCUSSION: A comprehensive search of the Medline database revealed only a few documented cases of glioependymal cysts (fewer than 30 cases). Remarkably, the majority (if not all) were located laterally rather than in the midline of the brain. Endoscopic fenestration and biopsy are effective and confirm the diagnosis. CONCLUSION: This instance of a rare glioependymal cyst located in the midline, spanning the suprasellar and retrosellar regions, is an uncommon occurrence. Its sole presentation was precocious puberty. The successful management of this condition was achieved through an endoscopic approach, leading to the normalization of endocrine abnormalities.

Glomangiomyoma of the Stomach: Case Report.

Glomus tumor (glomus cell tumor) is a rare, often benign neoplasm, which is, in most cases, seen as a solitary bluish nodule involving the nail beds. Solid glomus tumor, glomangioma, and glomangiomyoma are the three main histopathological variants. In this case report, we present the rarest subtype of a glomus tumor, glomangiomyoma, in an atypical location: the stomach. A 45-year-old female from Syria presented to the clinic with severe dizziness and left epigastric abdominal pain accompanied by melena. We performed a thorough clinical study, laboratory workup, upper gastrointestinal endoscopy, endoscopic ultrasound, CT scan, as well as macroscopic and microscopic histologic examination of the surgical specimen, in addition to the immunohistochemical staining. Although rare, gastric glomangiomyoma was diagnosed and a 4.5 × 3 × 3 cm soft tissue mass was resected from the gastric antrum, and no clinical or endoscopic evidence of recurrence was observed for the follow-up duration of 4 years. Undiagnosed gastric lesions with unexplained symptoms should be further investigated and not be disregarded immediately. To the best of our knowledge, this is only the second report of an instance of gastric glomangiomyoma.

Primary non-Hodgkin lymphoma of the chiasm and optic tract in a nonimmunocompromised patient: illustrative case.

BACKGROUND: Chiasmatic and optic track lymphoma as the primary lesion of the central nervous system (CNS) is extremely rare. OBSERVATIONS: The authors report a case of a previously healthy 62-year-old woman who presented with quick and progressive visual impairment leading to bilateral blindness. Brain imaging studies suggested glioma or lymphoma of the chiasm and the posterior visual pathway. Postoperative examination revealed low-grade malignant B-cell lymphoma. No evidence of extracranial lymphoma was found, so a final diagnosis of primary CNS lymphoma (PCNSL) was made. LESSONS: To the authors' knowledge, PCNSL confined to the optic chiasm has rarely been reported in nonimmunocompromised patients. The present case of lymphoma affecting the optic chiasm and optic tract is extremely rare.

Primary central nervous system Hodgkin Lymphoma: A case discussion and a hypothesis on the etiology.

Hodgkin Lymphoma (HL) is a systemic disease with involvement of the cervical, supraclavicular, and mediastinal lymph nodes. It is commonly diagnosed in patients within the second and third decades of their lives. Diagnosis is usually made based on the distinct morphological and immunohistochemical characteristics, with the tissue biopsy being the cornerstone of workup. Extranodal presentation of HL is unusual and seldom encountered. Primary HL of the central nervous system (CNS) is exceedingly rare. We herein report a case of a 38-year-old male patient who was diagnosed with primary CNS-HL. The patient was treated with complete surgical resection followed by radiotherapy and chemotherapy. The patient was disease-free for 7 years postoperatively without any clinical evidence of relapse. We also discussed a possible role of CNS regulatory T-cells (Tregs) in developmental primary CNS-HL.

Mixed corticomedullary adrenal carcinoma - case report: Comparison in features, treatment and prognosis with the other two reported cases.

INTRODUCTION: Adrenal corticomedullary adenoma was reviewed in many cases in PubMed Library, While the coincidence corticomedullary adrenal carcinoma in the same gland was just described in two cases in the medical literature. Our case is the third to be reported and was treated with surgery and adjuvant chemotherapy and followed for two years. PRESENTATION OF CASE: A 50-year-old man suffered from a mass effect in the left abdominal side. While the laboratory showed a mild elevation in the levels of both serum cortisol and 24h urine cortisol, radiological images were highly suggested an adrenal malignant tumor without metastasis. At surgery a 22cm sized mass was completely resected. Immunohistochemical study identified expression of both adrenocortical carcinoma and pheochromocytoma markers. DISCUSSION: Cases of coincidence corticomedullary tumor have been published in many reviews, cortical and/or medulllary hypersecretion were not always detected preoperatively by biochemical tests. Mixed corticomedullary carcinoma are exceedingly rare, we came across three reported cases in medical literature, in one case laboratory tests confirmed both cortical and medulla hypersecretion, while the two others detected only cortical hypersecretion. The final diagnosis was always confirmed by immunohistochemical staining. CONCLUSION: It could be noted that this is the first comparison of presentation, diagnosis, treatments and follow-up of the three cases of Mixed corticomedullary carcinoma. This could contribute to understanding the behavior and management of this rare malignancy and make it more familiar in clinical practice.

Analysis of 92 mitral pulmonary autograft replacement (Ross II) operations.

OBJECTIVE: The study objective was to find a mitral valve substitute that does not require lifelong anticoagulation and is not affected by tissue degeneration in the long term. METHODS: Between July 14, 1997, and August 8, 2004, a total of 92 patients with irreparable mitral valve disease underwent mitral valve replacement with the pulmonary autograft encased within a Dacron tubing for support. In 4 patients, the autograft had to be sacrificed at the initial operation. Of the remaining 88 patients, 62 were female, and the age ranged from 4 to 64 years (mean 39 years). Eighty-six patients had rheumatic mitral disease, and 2 patients had congenital mitral disease. RESULTS: Operative transesophageal echocardiography initially showed adequate valve characteristics (mean valve area 2.8 cm2, mean gradient 3.9 mm Hg, no significant regurgitation) in all 88 patients. Operative mortality was 4.6%, and late mortality definitely related to the operation was 7.9%. Four patients were lost to follow-up; the mean follow-up was 60 months. Progressive regurgitation and stenosis developed in 9 patients over 2 to 5 years, 4 of whom had their grafts explanted. The autograft was explanted in 1 patient because of endocarditis. Mild pulmonic stenosis developed in 3 patients, and critical pulmonic stenosis developed in 1 patient. At 5 years follow-up, freedom from degeneration was 93.4%, freedom from reoperation was 94.2%, and freedom from all death was 86.0%. CONCLUSION: Although the Ross II operation is difficult and harbors significant risk, it remains an option for patients with irreparable mitral disease who have a long life expectancy and who cannot be placed on lifelong anticoagulation.