Cases reported "Germinoma"

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1/44. De novo development of a cavernous malformation of the spinal cord following spinal axis radiation. Case report.

    Analysis of recent reports has suggested that cavernous malformations (CMs) of the brain may have an acquired pathogenesis and a dynamic pathophysiological composition, with documented appearance of new lesions in familial cases and following radiotherapy. The authors report the first case of demonstrated de novo formation of an intramedullary CM following spinal radiation therapy. A 17 year-old boy presented with diabetes insipidus and delayed puberty. Evaluation of endocrine levels revealed hypopituitarism, and magnetic resonance (MR) imaging demonstrated an infundibular mass. The patient underwent a pterional craniotomy and removal of an infundibular germinoma. The MR image of the spine demonstrated normal results. The patient received craniospinal radiation therapy and did well. He presented 5 years later with acute onset of back pain, lower-extremity weakness and numbness, and difficulty with urination. An MR image obtained of the spine revealed an intramedullary T-7 lesion; its signal characteristics were consistent with a CM. The patient was initially managed conservatively but developed progressive myelopathy and partial brown-sequard syndrome. Although he received high-dose steroids and bed rest, his symptoms worsened. He underwent a costotransversectomy and excision of a hemorrhagic vascular lesion via an anterolateral myelotomy. Pathological examination confirmed features of a CM. The patient has done well and was walking without assistance within 4 weeks of surgery. De novo genesis of CMs may be associated with prior radiation therapy to the spinal cord.
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2/44. A rare case of extramedullary spinal cord germinoma.

    A rare case of spinal cord germinoma in a 20-year-old Japanese female is presented. The tumor was detected at the level of T11 and L3 by myelography. Histological findings of the resected tumor were identical to germinoma, which is characterized by polygonal tumor cells, lymphocytic infiltrate, and granuloma. Tumor cells and lymphocytes reacted with antibodies for placental alkaline phosphatase and CD45, respectively. A few multinucleated giant cells, negative immunohistochemically for human chorionic gonadotropin were also seen. The present case is the 10th case of spinal cord germinoma and is thought to be the first case of such a tumor located extramedullary. diagnosis of such a rare tumor in an unusual location needs proper histological, ultrastructural and immunohistochemical evaluation.
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3/44. Metastatic spinal intramedullary germinoma with elevated cerebrospinal fluid chorionic gonadotropin: a case report.

    We treated a patient whose unusual recurrent germinoma illustrates the diagnostic value of measuring human chorionic gonadotropin beta subunit (HCG-beta) in cerebrospinal fluid (CSF) and serum. A 25-year-old man with a suprasellar germinoma and ventricular dissemination was treated successfully with systemic chemotherapy and cranial irradiation. Six years later he developed progressive numbness and weakness in both upper extremities. magnetic resonance imaging (MRI) disclosed an intramedullary spinal cord tumor in the cervical region. The CSF concentration of HCG-beta was elevated and exceeded that in serum. After completion of systemic chemotherapy and spinal irradiation, symptoms subsided and the tumor was no longer evident on MRI. Based on the patient's history and the rapid response of the tumor to treatment, the spinal cord tumor was considered a metastatic intramedullary spinal germinoma representing CSF dissemination via the central canal.
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4/44. Primary spinal germinoma in a patient with concomitant Klinefelter's syndrome.

    It is known that patients with Klinefelter's syndrome are inclined to develop concomitant malignant tumours, as well as extragonadal germ cell tumours. The association of a primary spinal germinoma in a patient with Klinefelter's syndrome is reported for the first time, and the coincidence of elevated gonadotropin levels and oncogenesis is discussed.
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5/44. Metastatic testicular cancer presenting as spinal cord compression: report of two cases.

    BACKGROUND: Testicular cancers are heterogenous neoplasms often found in young adults. They tend to metastasize to the chest, retroperitoneum, or neck, but rarely to the long bones or skeleton. However, they can cause neurologic compromise and should be considered in young male patients who present with symptoms of a spine lesion and no known primary cancer. methods: Two patients presented with back pain and a rapid progression of lower extremity weakness. Both underwent radiographic workup and emergency surgery. Metastatic workup revealed testicular cancer and widespread metastases. RESULTS: Both patients improved neurologically after surgery, but neither regained the ability to ambulate independently. They both underwent chemotherapy. One patient is alive at 1 year follow-up; the other died 9 months after surgery of widespread metastases. CONCLUSIONS: Vertebral metastases from testicular tumors, although rare, should be considered in young men presenting with spinal cord compression. work-up should include magnetic resonance imaging (MRI) of the spine and computed tomography (CT) of the chest, abdomen, and pelvis. Urgent intervention may be required, as these two cases show that loss of neurologic function can be rapid and permanent.
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6/44. De novo spinal teratoma after treatment of an intracranial germ cell tumor.

    The authors report an extremely rare case of de novo spinal teratoma after treatment for intracranial germ cell tumor. A 17-year-old male developed pain of bilateral lower extremities and urinary retention 18 months after complete remission of intracranial mixed germ cell tumor. magnetic resonance imaging revealed a huge spinal tumor associated with spina bifida occulta. Total resection was performed, and histogenetical findings led to the diagnosis of a mature teratoma with normal p16 gene, whereas analysis of intracranial tumor showed p16 deletion. The spinal anomaly and genetic analysis strongly suggest that the spinal teratoma was a de novo tumor rather than a metastasis or dissemination of the original intracranial germ cell tumor.
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7/44. Intrathecal and intraperitoneal germinomas occurring 20 years after total removal of a pineal teratoma. Case report.

    In 1980, a 10-year-old boy was admitted to the authors' hospital with consciousness disturbance and vomiting. Computerized tomography (CT) scans revealed a pineal mass lesion and hydrocephalus. The tumor was totally removed and a ventriculoperitoneal (VP) shunt was inserted. Histological investigation of a surgical specimen revealed that it was a teratoma. Five years later, the patient was readmitted to the same institution with polyuria. Magnetic resonance (MR) imaging revealed a mass lesion in the suprasellar region. The patient received systemic chemotherapy, and the tumor disappeared; however, 2 years after the chemotherapy, MR imaging demonstrated a right lateral ventricular mass. The tumor was totally resected, and histopathological investigation revealed a teratocarcinoma. Three years after the chemotherapy, CT scanning revealed suprasellar and right lateral ventricular tumor recurrences, for which the patient received irradiation and chemotherapy. The tumors disappeared and the patient achieved complete remission that lasted longer than 10 years. On January 25, 2000, however, he noticed hip pain. Lumbar MR imaging demonstrated a spinal tumor below L-4 and also an abdominal tumor. The abdominal tumor was totally removed, and the histological findings identified it as a germinoma. The patient received systemic chemotherapy and the tumor disappeared completely. The authors believe that the suprasellar tumor was a metachronous germinoma and that it had metastasized through the intrathecal route and the VP shunt.
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8/44. Delayed surgical resection of central nervous system germ cell tumors.

    OBJECTIVE: To determine the value of delayed surgical resection in patients with central nervous system germ cell tumors who exhibit less than complete radiographic response despite declining serum and cerebrospinal fluid (CSF) tumor markers after initial chemotherapy. methods: We retrospectively analyzed 126 patients enrolled on two international multicenter clinical trials (the First and Second International central nervous system Germ Cell Tumor Studies) for patients with newly diagnosed central nervous system germ cell tumors. After at least three cycles of chemotherapy, 10 of these patients underwent delayed surgical resection owing to evidence of residual radiographic abnormalities despite declining or completely normalized serum and CSF levels of alpha-fetoprotein and human chorionic gonadotropin. RESULTS: Eight of these patients demonstrated nongerminomatous germ cell tumor elements at the time of initial diagnosis. In these patients, either serum or CSF tumor markers were elevated initially. Two patients demonstrated pure germinomas with normal levels of serum and CSF tumor markers. After chemotherapy, radiographic evaluation revealed a partial response in seven patients, a minor response in one patient, and stable disease in two patients. All 10 patients had either normal or decreasing levels of serum and CSF tumor markers before second-look surgery. At delayed surgical resection, 7 of the 10 patients underwent gross total resection, and 3 patients underwent subtotal resection of residual lesions. Pathological findings at second-look surgery demonstrated three patients to have mature teratomas, two with immature teratomas, and five with necrotic or scar tissue alone. To date, 7 of the 10 patients have had no recurrence during an average follow-up time of 36.9 months (range, 3-96 mo). Three of four patients with nongerminomatous germ cell tumors who had tumor markers that were decreased, but not normalized, before second-look surgery eventually developed tumor dissemination/progression, and they required subsequent radiation therapy despite having teratoma or necrosis/scar tissue at delayed surgery. In contrast, three of four patients with nongerminomatous germ cell tumors and completely normalized markers did not progress and did not require radiation therapy. CONCLUSION: Delayed surgical resection should be considered in patients with central nervous system germ cell tumors who have residual radiographic abnormalities and normalized tumor markers, because these lesions are likely to be teratoma or necrosis/scar tissue. However, second-look surgery should be avoided in patients whose tumor markers have not normalized completely.
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9/44. CNS germinoma in a boy with simple virilizing 21-hydroxylase deficiency and precocious puberty.

    A 6 year-old boy presented with peripheral precocious puberty and was diagnosed as having simple virilizing 21-hydroxylase deficiency based on clinical features and elevated 17-hydroxyprogesterone levels on ACTH stimulation. He was managed with glucocorticoids and mineralocorticoids. Two years later he presented with features of CNS involvement in the form of seizures and raised intracranial pressure with rapid progression of puberty. Contrast enhanced CT scan of brain showed an intraventricular tumor with cerebrospinal fluid cytology suggestive of germinoma. serum and CSF levels of human chorionic gonadotropin (hCG) and alphafetoprotein (AFP) were elevated, confirming the diagnosis of germinoma.
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10/44. Intramedullary spinal cord germinoma: case report and review of the literature.

    A 33-year-old man with an intramedullary spinal cord germinoma was treated successfully with partial resection, combined chemotherapy with carboplatin and etoposide, and reduced-dose local-field radiation therapy. The authors believe this is the first case without syncytiotrophoblastic giant cells so treated. The literature is reviewed, and the appearance, radiologic and histopathologic features, and treatment of germinomas are discussed.
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