The authors report on 17 patients having primary central nervous system(CNS) lymphomas between January, 1981 and August, 1992. All patients were treated at Seoul National University Hospital. Only three cases underwent surgery only and fourteen cases underwent surgery and radiation theraphy with or without chemotherapy. There were no immunosuppressive patients. Sixteen patients were analyzed for survival studies, because one case was lost during follow-up. The median survival time(MST) was 26 months and one- and two-year survival rates for the 16 patients were 87.5% and 65.6% respectively. The rate of survival was analyzed according to possible prognostic factors factors;age and sex, multiplicity and location of tumors, preoperative Karnofsky performance score(KPS), pathological subclassification, type of surgery, preoperative steroid therapy, postoperative adjuvant therapy, extent of radiation, chemotherapy and the degree of response to treatment at three months follow-up. Postoperative adjuvant therapy and the degree of response to the treatment correlated with survival. Fourteen patients who underwent postoperative adjuvant therapy had a median survival time of 63 months. The one- and two-year survival rates were 100% and 75% respectively compared to the MST of 1.5 months with survival rates of 0% and 0% retrospectively for the two patients who did not. A higher long term survival rate was observed in the group with better immediate responses. In nine of 16 patients with complete responses, the MST, one-, and two-year survival rates were 63 months, 100%, and 83% respectively. The authors suggest that preoperative application of steroids may be considered in case of clinically suspected primary CNS lymphomas. It is concluded that after obtaining the tissue diagnosis of primary CNS lymphomas, radiotherapy with or without chemotherapy should be performed until the complete response.
Brain Neoplasms ; Central Nervous System* ; Diagnosis ; Drug Therapy ; Follow-Up Studies ; Humans ; Lymphoma ; Radiotherapy ; Retrospective Studies ; Seoul ; Steroids ; Survival Rate*

PURPOSE: Diencephalic syndrome is an uncommon cause of failure to thrive in early childhood that is associated with central nervous system neoplasms in the hypothalamic-optic chiasmatic region. It is characterized by complex signs and symptoms related to hypothalamic dysfunction; such nonspecific clinical features may delay diagnosis of the brain tumor. In this study, we analyzed a series of cases in order to define characteristic features of diencephalic syndrome. METHODS: We performed a retrospective study of 8 patients with diencephalic syndrome (age, 5-38 months). All cases had presented to Seoul National University Children's Hospital between 1995 and 2013, with the chief complaint of poor weight gain. RESULTS: Diencephalic syndrome with central nervous system (CNS) neoplasm was identified in 8 patients. The mean age at which symptoms were noted was 18+/-10.5 months, and diagnosis after symptom onset was made at the mean age of 11+/-9.7 months. The mean z score was -3.15+/-1.14 for weight, -0.12+/-1.05 for height, 1.01+/-1.58 for head circumference, and -1.76+/-1.97 for weight-for-height. Clinical features included failure to thrive (n=8), hydrocephalus (n=5), recurrent vomiting (n=5), strabismus (n=2), developmental delay (n=2), hyperactivity (n=1), nystagmus (n=1), and diarrhea (n=1). On follow-up evaluation, 3 patients showed improvement and remained in stable remission, 2 patients were still receiving chemotherapy, and 3 patients were discharged for palliative care. CONCLUSION: Diencephalic syndrome is a rare cause of failure to thrive, and diagnosis is frequently delayed. Thus, it is important to consider the possibility of a CNS neoplasm as a cause of failure to thrive and to ensure early diagnosis.
Astrocytoma ; Brain Neoplasms ; Central Nervous System ; Central Nervous System Neoplasms ; Diagnosis ; Diarrhea ; Drug Therapy ; Early Diagnosis ; Failure to Thrive* ; Follow-Up Studies ; Head ; Humans ; Hydrocephalus ; Hypothalamic Diseases ; Infant* ; Optic Nerve Glioma ; Palliative Care ; Retrospective Studies ; Seoul ; Strabismus ; Vomiting ; Weight Gain

Primary central nervous system marginal zone B-cell lymphoma (MZBCL) is very rare, with only a few reported cases worldwide. It has an indolent disease course with high cure potential. We experienced a rare case of dural MZBCL of mucosa-associated lymphoid tissue (MALT) in a 69-year-old man who presented with headache. A magnetic resonance imaging scan of brain showed a 1.9×3.6-cm-sized extra-axial mass with a broad based dural attachment to the anterosuperior aspect of the falx cerebri, radiographically consistent with meningioma. Surgical resection yielded a MZBCL of the MALT type. Histopathology revealed a lymphoplasmacytic infiltration of the dura, and immunohistochemical study showed a B-cell phenotype with CD20, bcl-2, MUM-1, Ki-67 positive. He was treated with chemotherapy after complete surgical resection and remained free of disease at 30 months after chemotherapy. MALT lymphoma must be considered in the differential diagnosis in patients presenting radiographically with meningioma.
Aged ; B-Lymphocytes ; Brain ; Central Nervous System ; Central Nervous System Neoplasms ; Diagnosis, Differential ; Drug Therapy ; Dura Mater ; Headache ; Humans ; Lymphoid Tissue ; Lymphoma, B-Cell, Marginal Zone* ; Magnetic Resonance Imaging ; Meningioma ; Phenotype

Primary central nervous system marginal zone B-cell lymphoma (MZBCL) is very rare, with only a few reported cases worldwide. It has an indolent disease course with high cure potential. We experienced a rare case of dural MZBCL of mucosa-associated lymphoid tissue (MALT) in a 69-year-old man who presented with headache. A magnetic resonance imaging scan of brain showed a 1.9×3.6-cm-sized extra-axial mass with a broad based dural attachment to the anterosuperior aspect of the falx cerebri, radiographically consistent with meningioma. Surgical resection yielded a MZBCL of the MALT type. Histopathology revealed a lymphoplasmacytic infiltration of the dura, and immunohistochemical study showed a B-cell phenotype with CD20, bcl-2, MUM-1, Ki-67 positive. He was treated with chemotherapy after complete surgical resection and remained free of disease at 30 months after chemotherapy. MALT lymphoma must be considered in the differential diagnosis in patients presenting radiographically with meningioma.
Aged ; B-Lymphocytes ; Brain ; Central Nervous System ; Central Nervous System Neoplasms ; Diagnosis, Differential ; Drug Therapy ; Dura Mater ; Headache ; Humans ; Lymphoid Tissue ; Lymphoma, B-Cell, Marginal Zone* ; Magnetic Resonance Imaging ; Meningioma ; Phenotype

We report a case of primary central nervous system(CNS) lymphoma in an organ recipient. A 33-years-old man who underwent a renal transplantation 3years previously presented with headache and vomiting. In Brain computed tomography scans and magnetic resonance images showed multiple periventricular cystic rim enhancing masses. Pathologic diagnosis by stereotactic biopsy revealed malignant non-Hodgkins B-cell lymphoma. After pathologic confirmation, methotrexate chemotherapy and whole brain radiation therapy were done. Having experienced such a case, the authors strongly recommend to add primary CNS lymphoma as one of the differential diagnoses to brain abscess, metastatic brain tumor and glioblastoma multiforme in cases of multiple ring enhancing periventricular lesions of immunocompromised patient or organ recipient.
Biopsy ; Brain ; Brain Abscess ; Brain Neoplasms ; Central Nervous System* ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Glioblastoma ; Headache ; Immunocompromised Host ; Kidney Transplantation ; Lymphoma* ; Lymphoma, B-Cell ; Methotrexate ; Vomiting

Central nervous system atypical teratoid/rhabdoid tumor (AT/RT) of infancy and childhood, a new entity among malignant pediatric brain tumors, is unique histologic entity with an extremely aggressive natural history. Histologically AT/RT is defined as a polymorphous neoplasm often featuring rhabdoid, primitive neuroectodermal tumor, epithelial, and mesenchymal components. AT/RT occurs mainly in the posterior fossa, so mimics medulloblastoma. AT/RT is characterized by the cytogenetic finding of monosomy 22 rather than i (17q). Standard chemotherapy for infant and childhood medulloblastoma, for which this entity is often mistaken, has been ineffective. Most children survive less than 12 months (mean survival: 8.5 months) after diagnosis. We report two cases of central nervous system AT/RT in young children, one of them is alive so far with multimodal treatment.
Brain Neoplasms ; Central Nervous System* ; Child ; Combined Modality Therapy ; Cytogenetics ; Diagnosis ; Drug Therapy ; Humans ; Infant ; Medulloblastoma ; Monosomy ; Natural History ; Neuroectodermal Tumors, Primitive

Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a germ cell tumor. A 29-year-old woman presented with polyuria, polydipsia and amenorrhea. Laboratory findings revealed hypopituitarism with central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on magnetic resonance imaging. Under the clinical impression of a germ cell tumor, the patient was treated with germ cell tumor chemotherapy (cisplatin and etoposide) and radiation therapy without biopsy. After initial shrinkage of the lesions, further growth of the tumor was observed and a biopsy was performed. The histopathology revealed LCH. After chemotherapy according to the LCH III protocol, the tumor disappeared. She is on regular follow up for 5 years without relapse. The present findings indicate that LCH should be included in the differential diagnosis of a suprasellar mass, even in adults, especially when it manifests with diabetes insipidus. This case also underscores the importance of a histopathologic diagnosis in patients with suprasellar tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive of a specific diagnosis.
Adult ; Amenorrhea ; Biopsy ; Central Nervous System Neoplasms ; Diabetes Insipidus ; Diabetes Insipidus, Neurogenic ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Female ; Follow-Up Studies ; Germinoma ; Histiocytosis, Langerhans-Cell* ; Humans ; Hypopituitarism ; Magnetic Resonance Imaging ; Neoplasms, Germ Cell and Embryonal ; Polydipsia ; Polyuria ; Recurrence ; Sella Turcica

Langerhans cell histiocytosis (LCH) has diverse clinical manifestations, including intracranial mass lesions. We report a case of LCH that manifested as a suprasellar mass, and initially misdiagnosed as a germ cell tumor. A 29-year-old woman presented with polyuria, polydipsia and amenorrhea. Laboratory findings revealed hypopituitarism with central diabetes insipidus, and a suprasellar mass and a pineal mass were observed on magnetic resonance imaging. Under the clinical impression of a germ cell tumor, the patient was treated with germ cell tumor chemotherapy (cisplatin and etoposide) and radiation therapy without biopsy. After initial shrinkage of the lesions, further growth of the tumor was observed and a biopsy was performed. The histopathology revealed LCH. After chemotherapy according to the LCH III protocol, the tumor disappeared. She is on regular follow up for 5 years without relapse. The present findings indicate that LCH should be included in the differential diagnosis of a suprasellar mass, even in adults, especially when it manifests with diabetes insipidus. This case also underscores the importance of a histopathologic diagnosis in patients with suprasellar tumors before the initiation of a specific therapy, even if the clinical findings are highly suggestive of a specific diagnosis.
Adult ; Amenorrhea ; Biopsy ; Central Nervous System Neoplasms ; Diabetes Insipidus ; Diabetes Insipidus, Neurogenic ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Female ; Follow-Up Studies ; Germinoma ; Histiocytosis, Langerhans-Cell* ; Humans ; Hypopituitarism ; Magnetic Resonance Imaging ; Neoplasms, Germ Cell and Embryonal ; Polydipsia ; Polyuria ; Recurrence ; Sella Turcica

PURPOSE: Leptomeningeal carcinomatosis occurs in about 5% of patients with solid tumor and is being diagnosed with increasing frequency as patients live longer and as neuro-imaging studies improve. In general, the most commom cancers that involved the leptomeninges are breast cancer, lung cancer, and malignant melanoma. MATERIALS AND METHODS: We investigated 25 patients presented with multiple neurologic symptoms and signs who were diagnosed with leptomeningeal carcinomatosis at the Yonsei Cancer Center from January 1990 to December 1999. RESULTS: The primary disease of leptomeningeal carcinomatosis were stomach cancer (10 cases), breast cancer (7 cases), lung cancer (5 cases), unknown primary cancer (2 cases) and common bile duct cancer (1 case). All patients were presented with multiple neurologic symptoms and signs involving the central nervous system (CNS), cranial nerve or spinal nerves. Twenty-one of twenty- five patients were treated with intrathecal chemotherapy, radiotherapy, or combination therapy. Fourteen of them (66.7%) experienced improvement or stabilization of neurologic symptom and sign. The median survival was 122 days (10-2190). CONCLUSION: In conclusion, although early diagnosis and active treatment of leptomeningeal carcinomatosis may improve the quality of life in selected patients, the median survival was relatively short. Therefore, new diagnostic and therapeutic strategy for leptomeningeal carcinomatosis were needed.
Breast Neoplasms ; Central Nervous System ; Common Bile Duct ; Cranial Nerves ; Drug Therapy ; Early Diagnosis ; Humans ; Lung Neoplasms ; Melanoma ; Meningeal Carcinomatosis* ; Neurologic Manifestations ; Quality of Life ; Radiotherapy ; Spinal Nerves ; Stomach Neoplasms

We report here the case of a suprasellar cystic germ cell tumor (GCT) initially diagnosed as an arachnoid cyst. A 10-year-old boy experienced headache, dizziness, and diplopia, and was shown to have an approximately 2 cm suprasellar cyst. Two months after endoscopic third ventriculostomy was performed, a 5-6 cm cystic mass with an internal enhancing component was observed in the suprasellar cistern. Serum human chorionic gonadotropin levels were slightly increased in the serum and cerebrospinal fluid (55 and 162 IU/L, respectively) but were strikingly elevated in the cystic fluid (14,040 IU/L). The patient showed complete remission, with only a very small cystic lesion remaining after surgery, chemotherapy, and radiation treatment for a suprasellar mixed GCT. However, follow-up after treatment was complicated by moyamoya syndrome and cerebral infarction. GCT can be considered as a rare differential diagnosis in the case of a suprasellar cystic mass. Evaluation of tumor markers and close follow-up will be necessary.
Arachnoid* ; Central Nervous System Cysts* ; Cerebral Infarction ; Cerebrospinal Fluid ; Child ; Chorionic Gonadotropin ; Diagnosis, Differential ; Diplopia ; Dizziness ; Drug Therapy ; Follow-Up Studies ; Germ Cells* ; Headache ; Humans ; Male ; Moyamoya Disease ; Neoplasms, Germ Cell and Embryonal* ; Biomarkers, Tumor ; Ventriculostomy