Case Report: Esthesioneuroblastoma Involving the Optic Pathways.

Olfactory neuroblastoma, or esthesioneuroblastoma, is an uncommon malignant tumor originating from the neural crest that commonly occurs in the upper nasal cavity. Its ectopic origin is extremely rare, especially when located in the optical pathways. This paper reports the case of a giant ectopic esthesioneuroblastoma of the optic pathways that were surgically treated through a cranio-orbital-zygomatic (COZ) craniotomy with extensive resection, in addition to a literature review. The patient is a 46-year-old female presenting with a 4-month history of visual loss in the left eye. Since she was previously blind in the right eye from a traumatic injury, it was evolving to loss of bilateral vision. Imaging depicted an expansive infiltrating lesion involving the entire path of the right optic nerve, extending to the optic chiasm, cisternal portion of the left optic nerve, bilateral optic tract, and hypothalamus. Investigation of pituitary function was unremarkable. Esthesioneuroblastoma is a rare tumor with poorly defined standard clinical management. Its ectopic presentation makes the diagnosis even more challenging, making it difficult to manage these cases properly. Surgeons should be aware of this rare possibility, as early aggressive treatment is likely to be associated with better results.

Primary ectopic Merkel cell carcinoma without skin involvement-report of a case with prolonged survival.

Merkel cell carcinoma is a rare neuroendocrine malignancy that arises from the dermis, in cases of immunocompromised, middle-aged patients or on skin exposed to sunlight. It only rarely presents in extra-cutaneous locations. We present the case of a 63-year-old female with a mass in the adipose tissue of the upper arm, without skin involvement and concurrent axillary lymph node enlargement. She was treated with wide excision and lymph node dissection; pathology led to the diagnosis of Merkel cell carcinoma, and she was subsequently submitted to adjuvant radiotherapy. No signs of recurrence are present 8 years postoperatively. Primary Merkel cell carcinoma can rarely be located in the adipose tissue without skin involvement. In cases of high suspicion, preoperative MRI scan can show the extent of the lesion, as well as lymph metastases.

Adenoma hipofisario ectópico del seno esfenoidal persistente tras cirugía manejado con tratamiento conservador / Ectopic pituitary adenoma in the sphenoid sinus persistent after surgery managed with conservative treatment

Los adenomas hipofisarios ectópicos (EPA) constituyen un reto diagnóstico, dada su escasa prevalencia y variada presentación en la que puede incluirse un síndrome de hipersecreción de hormonas hipofisarias. La clínica suele ser larvada e inespecífica, no presentan ninguna característica radiológica diferencial y el diagnóstico habitualmente es anatomopatológico. Sin embargo, a pesar de ser tumores benignos, pueden presentar un comportamiento agresivo, con invasión ósea y difícil resección completa, por lo que un diagnóstico de sospecha precoz podría resultar en un tratamiento más eficaz y con un menor número de complicaciones. Presentamos el caso de una paciente con un adenoma hipofisario ectópico silente en el seno esfenoidal con inmunohistoquímica positiva para Hormona de crecimiento (GH) y prolactina que presentaba restos tumorales tras la intervención quirúrgica y ha sido manejada con tratamiento médico conservado, con buenos resultados.

Ectopic pituitary adenomas constitute a diagnostic challenge, given their low prevalence and varied presentation in which a pituitary hormone hypersecretion syndrome may be included. Clinical symptoms are usually latent and nonspecific, they have no differential radiological characteristics and the diagnosis is usually anatomopathological. However, despite being benign tumors, they can exhibit aggressive behavior, with bone invasion and difficult complete resection, so a diagnosis of early suspicion could result in more effective treatment and fewer complications. We present the case of a patient with a silent ectopic pituitary adenoma in the sphenoid sinus with positive immunohistochemistry for Growth Hormone (GH) and prolactin who had tumor remnants after surgery and was managed with conservative medical treatment, with good results.

Massive thymoma of the mid-posterior mediastinum: an unprecedented case in a young adult / Timoma volumoso de mediastino medioposterior em adulto jovem: relato de caso inédito

Abstract We report an unprecedented case of ectopic thymoma in a young adult. A 33-year-old male presented with a 10-day history of non-productive cough and fever. Investigation revealed mediastinal widening without pulmonary involvement. Computed tomography showed a large mass-14.8 × 10.8 × 8.4 cm-in the mid-posterior mediastinum, and a biopsy obtained by video-assisted thoracoscopy indicated that the mass was a tumor. Immunohistochemistry showed combined thymoma type AB1. Because of the considerable proportions of the tumor and its close proximity to major structures, the patient was treated with chemotherapy.

Resumo Apresentamos caso inédito de volumoso timoma ectópico em adulto jovem. Homem de 33 anos de idade, encaminhado com tosse seca e febre diária havia 10 dias. Durante investigação observou-se alargamento mediastinal sem comprometimento pulmonar. Um tumor mediastinal medioposterior, medindo 14,8 × 10,8 × 8,4 cm, foi diagnosticado após tomografia computadorizada e biópsia videotoracoscópica. A imuno-histoquímica revelou timoma misto AB1. Devido à íntima relação com estruturas nobres e grandes proporções, optou-se pela quimioterapia.

Massive thymoma of the mid-posterior mediastinum: an unprecedented case in a young adult.

We report an unprecedented case of ectopic thymoma in a young adult. A 33-year-old male presented with a 10-day history of non-productive cough and fever. Investigation revealed mediastinal widening without pulmonary involvement. Computed tomography showed a large mass-14.8 × 10.8 × 8.4 cm-in the mid-posterior mediastinum, and a biopsy obtained by video-assisted thoracoscopy indicated that the mass was a tumor. Immunohistochemistry showed combined thymoma type AB1. Because of the considerable proportions of the tumor and its close proximity to major structures, the patient was treated with chemotherapy.

Apresentamos caso inédito de volumoso timoma ectópico em adulto jovem. Homem de 33 anos de idade, encaminhado com tosse seca e febre diária havia 10 dias. Durante investigação observou-se alargamento mediastinal sem comprometimento pulmonar. Um tumor mediastinal medioposterior, medindo 14,8 × 10,8 × 8,4 cm, foi diagnosticado após tomografia computadorizada e biópsia videotoracoscópica. A imuno-histoquímica revelou timoma misto AB1. Devido à íntima relação com estruturas nobres e grandes proporções, optou-se pela quimioterapia.

Possible esthesioneuroblastoma metastasis to paranasal sinuses: Clinical report and literature review.

BACKGROUND: Local recurrence, and regional and distant metastases, respectively, develop in 30% and 15% of patients with esthesioneuroblastoma (ENB). METHODS: This case report includes 3 patients with unusual recurrences of ENB in the sinonasal tract. RESULTS: Patient 1 developed ENB in the bilateral maxillary sinuses after unilateral endoscopic resection of a left ENB followed by postoperative proton radiotherapy. Patient 2 developed ENB at the left optic-carotid recess 7 years after a craniofacial resection and postoperative radiotherapy. Patient 3 developed ENB over the left zygomatic process of the maxilla 4 years after a cranioendoscopic resection and postoperative radiation therapy for ENB of the ethmoid sinuses with intracranial extension. The possibility of venous tumor emboli must be considered as a likely etiology. However, alternative explanations include the development of a second primary or potential transformation because of tumor signaling. CONCLUSION: We present 3 cases that may represent metastasis of ENB within the sinonasal tract.

Sphenoid esthesioneuroblastoma arising from the hindmost olfactory filament.

Esthesioneuroblastoma (ENB), or olfactory neuroblastoma, is a rare malignant neoplasm arising from the olfactory neuroepithelium. Typically, ENBs are found in the olfactory cleft with extension to the ethmoid sinuses or anterior skull base. Here we report a case of ENB located in the sphenoid sinus, which had been considered as an ectopic ENB. However, endoscopic resection revealed the continuity of the tumor with the hindmost olfactory filament. The present case suggests that an ENB in the sphenoid sinus was not ectopic, but arose from the normal olfactory neuroepithelium. This continuity of the ENB with this filament indicated that the tumor was not ectopic, and that there was possible tumor invasion into the olfactory neuroepithelium in the cribriform niche. Therefore, pathological examination of the olfactory neuroepithelium in the cribriform niche may be necessary in case of sphenoid ENBs.

Therapeutic potential of metformin in papillary thyroid cancer in vitro and in vivo.

Metformin, an anti-diabetic drug used in type 2 diabetes treatment, is reported to have oncopreventive or therapeutic roles in several human cancers. The present study investigated the therapeutic potential of physiologic dose of metformin in PTC. Metformin inhibited PTC cell viability and increased cell apoptosis in various doses (0.5-20mM) in BCPAP and BHP10-3SC cells. Western blot analysis demonstrated that the p-AMPK/AMPK ratio increased with increased metformin treatment. The ectopic tumor experiment was performed using BHP10-3SC cells and athymic nude mice. Oral metformin treatment via drinking water significantly delayed tumor growth in both tumor development model and established tumor models. Necrotic area in tumors significantly increased with metformin treatment. Western blot analysis revealed an increase in p-AMPK/AMPK ratio and suppressions of mTOR and Akt expressions in metformin-treated mice compared to the results in mock-treated control mice. Our results indicate that a physiologic dose of metformin has anti-tumorigenic effects that result from activation of AMPK signaling and inhibition of Akt signaling.

Primary Epidural Meningioma in Adolescence

Meningioma arises from the arachnoid cap cell found in the arachnoid layer of the meninges. Most meningiomas are located in the subdural space. Meningiomas are mostly found in adulthood between 20 and 60 years of age. A case of intracranial epidural meningioma without involvement of the subdural space is reported.

Ectopic Pituitary Adenoma within the Sphenoid Sinus

A 69-year-old woman presented with right abducent nerve paresis caused by an ectopic pituitary adenoma invading the posterior wall of the sphenoid sinus. The tumor was removed via transsphenoidal approach. The histological diagnosis was invasive pituitary adenoma with bony destruction. The symptom was improved without complication. The authors present a rare case of ectopic pituitary adenoma with a literature review.