Surg Neurol

Two cases of esophageal carcinoma metastatic to the pineal region with a review of the literature.

Schuster JM. Rostomily RC. Hahn C. Winn HR.
Department of Neurological Surgery, University of Washington Medical Center, Seattle 98104, USA.
BACKGROUND: Metastasis to the pineal region is rare, and there are no previously reported cases of esophageal carcinoma metastatic to the pineal region. CASE DESCRIPTION: We now present two cases of solitary esophageal carcinoma metastatic to the pineal region. In each case there was no evidence of disease progression at the primary site at the time of presentation, and neurologic symptoms were the first indication of recurrent disease. Both patients underwent infratentorial/supracerebellar resection of the pineal lesions and were subsequently referred for radiation therapy. CONCLUSIONS: These two cases emphasize that metastatic disease must be a likely differential consideration in a patient with a CNS lesion and a history of previous malignancy, even if the lesion is in an unusual location.

Colonic perforation by ventriculoperitoneal shunt tubing: a case of suspected silicone allergy.

Brownlee JD. Brodkey JS. Schaefer IK.
Department of Surgery, Columbia Saint Luke's Medical Center, Cleveland, OH 44104, USA.
BACKGROUND: A case of colonic perforation by a ventriculoperitoneal shunt is presented in a patient with several previous complications associated with shunt tubing. CASE DESCRIPTION: Initially managed by intravenous antibiotics, shunt externalization, and colonoscopy, the entire ventriculoperitoneal shunt system was subsequently replaced after cerebrospinal fluid cultures had grown Propionibacterium acnes and Streptococcus sanguis organisms. The patient has had three episodes of skin breakdown over his shunt tubing (two prior and one subsequent to colonic perforation) without evidence of shunt infection or malfunction. CONCLUSIONS: The etiology of these complications is consistent with silicone tubing allergy. Replacement with a polyurethane system produced no similar complications thus far, which further supports a possible silicone allergy to the ventriculoperitoneal shunt and possible etiology of this patient's colonic perforation.

Malignant ependymomas in a patient with Turcots syndrome: case report and management guidelines.

Year 1998
Mullins KJ. Rubio A. Myers SP. Korones DN. Pilcher WH.
Division of Neurological Surgery, Strong Memorial Hospital, University of Rochester School of Medicine, New York 14642-8670, USA.
BACKGROUND: Turcot's Syndrome is the association of multiple adenomatous polyps of the colon with a primary tumor of the central nervous system. We present the first reported case of Turcot's Syndrome in a patient with malignant ependymomas. Recent advances in the elucidation of the genetic basis for the hereditary forms of colon cancer have provided a clearer understanding of the etiology of Turcot's Syndrome. This new information is relevant to the neurosurgical community and provides updated guidelines in the diagnosis and management of patients with this complex disease process. RESULTS: Turcot's Syndrome is related to two distinct genetic errors. The first involves a germ-line mutation in the adenomatous polyposis coli (APC) gene, which is postulated to act as a tumor suppressor gene. The second is a germ-line defect in one of a group of genes responsible for DNA nucleotide mismatch repair. CONCLUSION: The elucidation of the gene defects responsible for the hereditary forms of colon cancer has provided a clearer understanding of the molecular basis of Turcot's Syndrome. Patients with hereditary forms of colon cancer and neurologic symptoms require immediate and thorough investigation because of their significantly increased risk of developing CNS tumors. Previously healthy patients diagnosed with a CNS tumor with a family history of adenomatous polyposis coli should undergo screening and surveillance colonoscopy as the CNS lesion may precede colonic symptoms. CNS screening guidelines for asymptomatic patients with adenomatous polyposis coli requires further risk analysis studies. All patients diagnosed with Turcot's Syndrome should be tested for the gene defect, including the CNS tumor tissue to provide further data on the genetic relationship between Turcot's Syndrome and the hereditary forms of colon cancer.

Medulloblastoma and glioblastoma multiforme in a patient with Turcot syndrome: a case report.

Year 1998
McLaughlin MR. Gollin SM. Lese CM. Albright AL.
Department of Neurological Surgery, Presbyterian University Hospital, University of Pittsburgh Medical Center, PA 15213-2582, USA.
BACKGROUND: Turcot syndrome (TS) or the glioma-polyposis syndrome, is a rare, heritable disorder thought by some authors to be a variant of familial adenomatous polyposis (FAP). It is characterized by central nervous system (CNS) neoplasms and gastrointestinal polyposis. METHODS: We present a case report of a patient who developed a medulloblastoma at age 5 years. Ten years later, she developed adenocarcinoma of the colon. Seven months after resection of this Dukes' C2 adenocarcinoma, she presented with a second primary CNS tumor, a glioblastoma multiforme. The patient's colonic adenocarcinoma and glioblastoma were evaluated histologically and cytogenetically. RESULTS: Cytogenetic analysis revealed the presence of chromosomal instability in both tumors. This unusual case of two primary CNS neoplasms in a patient with TS is presented with a review of the literature. CONCLUSIONS: The implications of the cytogenetic analysis are discussed in conjunction with the present knowledge of the molecular biology of TS.

Monstrous skull osteomas in a probable Gardners syndrome: case report.

Year 1998
Noterman J. Massager N. Vloeberghs M. Brotchi J.
Department of Neurosurgery, Hopital Universitaire Erasme, Brussels, Belgium.
BACKGROUND: Gardner's syndrome includes a clinical triad of familial polyposis coli, osteomas, and soft tissue tumors. METHODS: We present a very unusual case of probable isolated Gardner's syndrome characterized by extremely voluminous osteomas in the occipital and frontal areas associated with diffuse subcutaneous lipomas and without colic abnormality. RESULTS: The neurosurgical management included resection of the osteomas for cosmetic reasons. After a follow-up period of 5 years, the patient remains free of digestive complaints and the resected osteomas did not recur. CONCLUSIONS: The special clinical presentation of our case of possible Gardner's syndrome is discussed.

Primarily extracranial jugular foramen neurinoma manifesting with marked hemiatrophy of the tongue: case report.

Year 1998
Kanemoto Y. Ochiai C. Yoshimoto Y. Nagai M.
Department of Neurosurgery, Dokkyo University School of Medicine, Tochigi, Japan.
BACKGROUND: There have been no detailed descriptions of the clinical symptoms of a primarily extracranial jugular foramen neurinoma (JFN), because this type of tumor is extremely rare. CASE DESCRIPTION: Although the 51-year-old woman presented with only mild complaints of dysphagia and hoarseness, neurologic examination revealed marked left hemiatrophy of the tongue. Although magnetic resonance imaging suggested a JFN, the patient's mild symptoms and normal jugular foramen were potentially misleading in the diagnosis of this patient. Surgical exploration demonstrated that the tumor originated from the extracranial portion of the 10th cranial nerve, extending into the jugular foramen. Subtotal resection ameliorated the 12th cranial nerve palsy. CONCLUSION: The authors present a case of a primarily extracranial JFN manifesting as a 12th nerve palsy. The clinical symptoms and signs produced by a tumor in this extremely rare location are discussed.