Acute hydrocephalus due to a primary malignant peripheral nerve sheath tumor of the cervicothoracic junction: A case report and review of the literature.

BACKGROUND: The estimated incidence of malignant nerve sheath tumors is 0.001% per year, and only 2-3% of those tumors involve the spinal nerves. We present a rare case of acute hydrocephalus caused by primary malignant peripheral nerve sheath tumor of the cervicothoracic junction. CASE DESCRIPTION: A 29-year-old previously healthy male patient, except for a history of two previous surgeries for ulnar nerve entrapment and progressive left upper extremity weakness, presented with acute onset somnolence. The CT and MRI revealed hydrocephalus and periventricular edema. The patient underwent ventriculoperitoneal shunt surgery. Postoperative MRI of the spine revealed a 6×3×3cm intra-extradural lesion at C7-T1 level and multiple metastases in other spinal segments. The patient underwent combined surgical excision and the tumor was diagnosed as a malignant peripheral nerve sheath tumor based on pathological and immunohistological findings. Radiation therapy and chemotherapy were initiated. CONCLUSION: Primary malignant peripheral nerve sheath tumor of the spine is a very aggressive tumor with a very high recurrence rate, significant potential for metastasis and very poor overall prognosis. They may present with features of more frequent diseases, such as peripheral neuropathies and may be overlooked as in our case. Thus, suspected cases should undergo a more detailed examination.

Lung carcinoma metastasis presenting as a pineal region tumor.

The pineal region is an unusual site for brain metastasis and most metastatic pineal lesions are asymptomatic. A 53 year-old man presented with severe headache, limitation of upward gaze and diplopia. The patient's neurological examination was unremarkable. Magnetic resonance imaging (MRI) scans of the brain demonstrated a 1.5 x 2 cm well demarcated solitary mass in the pineal region with hydrocephalus. Surgery was performed and adenocarcinoma was diagnosed. A systemic investigation revealed adenocarcinoma of the lung as primary lesion. Although rare, metastatic tumor should be considered in the differential diagnosis of pineal region tumors.

Lung carcinoma metastasis presenting as a pineal region tumor

The pineal region is an unusual site for brain metastasis and most metastatic pineal lesions are asymptomatic. A 53 year-old man presented with severe headache, limitation of upward gaze and diplopia. The patient’s neurological examination was unremarkable. Magnetic resonance imaging (MRI) scans of the brain demonstrated a 1,5 x 2 cm well demarcated solitary mass in the pineal region with hydrocephalus. Surgery was performed and adenocarcinoma was diagnosed. A systemic investigation revealed adenocarcinoma of the lung as primary lesion. Although rare, metastatic tumor should be considered in the differential diagnosis of pineal region tumors (AU)

La región pineal es un sitio inusual para el depósito de metástasis y la mayoría de las metástasis pineales son asintomáticas. Un hombre de 53 años debutó con una cefalea intensa, limitación en la supraversión y diplopia. La exploración neurológica fue irrelevante. La resonancia magnética (RM) cerebral demostró una lesión solitaria de 1,5 x 2 cm bien delimitada, en la región pineal con hidrocefalia. Fue intervenido quirúrgicamente siendo el diagnóstico anatomopatológico de adenocarcinoma. Un estudio sistémico descubrió un adenocarcinoma de pulmón como tumor primario. Aunque muy raramente, las metástasis deben considerarse en el diagnóstico diferencial de tumores de la región pineal. %1 of all intracranial tumors27,30,32. Pineal region metastasis is even rarer and accounted for only 1.8% to 4% in literature3,8,22,29. In approximately half of reported cases, the pineal gland was the only site of intracranial metastasis with variable sizes1,2,16,17,25,28,33,35. The most common site of primary origin is lung carcinoma, followed by breast carcinoma and malignant melanoma33. The mechanism of pineal metastasis seems most probable via the bloodstream.Here we report a patient with a pineal region metastasis proven to be from adenocarcinoma of the lung mimicking a primary pineal region tumor (AU)