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: 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

A 43-year-old male presented with a painless left testicular mass. The pathologic diagnosis of the radical orchiectomy specimen was peripheral T-cell lymphoma, unspecified (PTCL-u). According to the Ann Arbor staging system, his initial stage was III because of the right nasopharyngeal involvement. After first-line chemotherapy with four courses of the CHOP regimen and this was followed by involved-field radiotherapy, he achieved complete remission. Two months later, disease recurred to the left ciliary body of the left eye without evidence of involvement at other sites. Although the patient received intensive chemotherapy with autologous hema-topoietic stem cell transplantation, he ultimately died of leptomeningeal seeding. Because both the central nervous system (CNS) and the orbit are sanctuary sites for chemotherapy, orbital infiltration of lymphoma should prompt physicians to evaluate involvement of the CNS and to consider performing prophylactic intrathecal chemotherapy as a treatment option.
Adult ; Central Nervous System ; Ciliary Body ; Diagnosis ; Drug Therapy ; Eye Neoplasms ; Humans ; Lymphoma ; Lymphoma, Non-Hodgkin ; Lymphoma, T-Cell* ; Lymphoma, T-Cell, Peripheral ; Male ; Orbit* ; Orchiectomy ; Radiotherapy ; Recurrence* ; Stem Cell Transplantation ; T-Lymphocytes* ; Testis

This retrospective study investigated the clinical characteristics and outcomes of second malignant neoplasms (SMNs) in survivors of childhood cancer from multiple institutions in Korea. A total of 102 patients from 11 institutions who developed SMN after childhood cancer treatment between 1998 and 2011 were retrospectively enrolled. The most common primary malignant neoplasms (PMNs) were central nervous system (CNS) tumors (n = 17), followed by acute lymphoblastic leukemia (n = 16), non-Hodgkin lymphoma (n = 13), and osteosarcoma (n = 12). The most common SMNs were therapy-related myeloid neoplasms (t-MNs; acute myeloid leukemia [AML], 29 cases; myelodysplastic syndrome [MDS], 12 cases), followed by thyroid carcinomas (n = 15) and CNS tumors (n = 10). The median latency period was 4.9 years (range, 0.5-18.5 years). Among 45 patients with solid tumors defined as an SMN, 15 (33%) developed the lesion in a field previously subjected to radiation. The 5-year overall survival (OS) rate of patients with an SMN was 45% with a median follow-up time of 8.6 years. Patients with AML, MDS, and CNS tumors exhibited the poorest outcomes with 5-year OS rates of 18%, 33%, and 32%, respectively, whereas those with second osteosarcoma showed comparable outcomes (64%) to patients with primary counterpart and those with second thyroid carcinoma had a 100% OS rate. Further therapeutic efforts are recommended to improve the survival outcomes in patients with SMNs, especially in cases with t-MNs and CNS tumors.
Adolescent ; Antineoplastic Agents/therapeutic use ; Central Nervous System Neoplasms/diagnosis/drug therapy/radiotherapy ; Child ; Child, Preschool ; Disease-Free Survival ; Hospitals ; Humans ; Infant ; Leukemia, Myeloid, Acute/diagnosis/epidemiology/mortality/therapy ; Myelodysplastic Syndromes/diagnosis/epidemiology/mortality/therapy ; Neoplasms, Second Primary/*diagnosis/epidemiology/mortality/therapy ; Osteosarcoma/diagnosis/epidemiology ; Retrospective Studies ; Stem Cell Transplantation ; Survival Rate ; Transplantation, Autologous ; Young Adult

This retrospective study investigated the clinical characteristics and outcomes of second malignant neoplasms (SMNs) in survivors of childhood cancer from multiple institutions in Korea. A total of 102 patients from 11 institutions who developed SMN after childhood cancer treatment between 1998 and 2011 were retrospectively enrolled. The most common primary malignant neoplasms (PMNs) were central nervous system (CNS) tumors (n = 17), followed by acute lymphoblastic leukemia (n = 16), non-Hodgkin lymphoma (n = 13), and osteosarcoma (n = 12). The most common SMNs were therapy-related myeloid neoplasms (t-MNs; acute myeloid leukemia [AML], 29 cases; myelodysplastic syndrome [MDS], 12 cases), followed by thyroid carcinomas (n = 15) and CNS tumors (n = 10). The median latency period was 4.9 years (range, 0.5-18.5 years). Among 45 patients with solid tumors defined as an SMN, 15 (33%) developed the lesion in a field previously subjected to radiation. The 5-year overall survival (OS) rate of patients with an SMN was 45% with a median follow-up time of 8.6 years. Patients with AML, MDS, and CNS tumors exhibited the poorest outcomes with 5-year OS rates of 18%, 33%, and 32%, respectively, whereas those with second osteosarcoma showed comparable outcomes (64%) to patients with primary counterpart and those with second thyroid carcinoma had a 100% OS rate. Further therapeutic efforts are recommended to improve the survival outcomes in patients with SMNs, especially in cases with t-MNs and CNS tumors.
Adolescent ; Antineoplastic Agents/therapeutic use ; Central Nervous System Neoplasms/diagnosis/drug therapy/radiotherapy ; Child ; Child, Preschool ; Disease-Free Survival ; Hospitals ; Humans ; Infant ; Leukemia, Myeloid, Acute/diagnosis/epidemiology/mortality/therapy ; Myelodysplastic Syndromes/diagnosis/epidemiology/mortality/therapy ; Neoplasms, Second Primary/*diagnosis/epidemiology/mortality/therapy ; Osteosarcoma/diagnosis/epidemiology ; Retrospective Studies ; Stem Cell Transplantation ; Survival Rate ; Transplantation, Autologous ; Young Adult

Tumors of the central nervous system are the second most frequent neoplasm in children. In a review of 43 children with brain tumor under the age of 16 evaluated at a pediatric and neurosurgical department at Severance Hospital Yonsei University during a 61/2 year period, following results were obtained. 1. All 43 cases were diagnosed by pathological examination as well as brain CT scan. The sex ratio of male and female was equal. The age distribution was highest between 13-14 year of age and 7% of them were under 1 year of age. 2. There was a small preponderance of infratentorial tumors (51%) over supratentoral tumors (49%). 3. Among 43 verified brain tumors by pathological examination, glioma represented 75%, of which astrocytoma was the commonest neoplasm being 19 cases (45%) and remainder were medulloblastoma 10 cases (23%), choroid plexus droglioma 2 cases (5%), ependymoma 1case (2%). The rest were craniopharyngioma 6 cases (14%), choroid plexus papilloma 1 case (2%), neuroblastoma 2 cases (5%), pineal teratoma 1 case (2%), melanotic neuroectodermal turmor 1 case (2%). 4. The most common symptom was headache occuring 63% of the patients followed by vomiting, motor weakness, visual disturbance, gait distrubance, mental disturbance and seizure in order of trequency. Neurological signs showed positive Babinski sign, papilledema, cerebellar sign, 6th nerve palsy and facial weakness. 5. Plain skull X-ray changes were noted in 26 out of the 43 cases (61%). Those were suture separation of skull noted 35% of tumors followed in frequency by increased digital marking, sella changes and calcification. 6. In brain CT scan studies, the most common abnormal finding was solid mass shadow followed by cystic mass shadow, solid and cystic mass shadow. After contrast infusion, diffuse enhancement was the most common features. 7. 27 cases were treated by operation only, 15 by operation and radiotherapy, 1 by operation, radiation and chemotherapy. 8. Progonsis of brain tumor was very poor. 5 of 43 cases died within 1 month of diagnosis, 1 year survival rate was 19%, and 2 year survival rate was 16%. The survival rate was better in the group of patients in whom total or subtotal resection combined with radiotherapy was performed. 53% of cases were not able to follow-up or discharged anainst advice without proper treatment.
Abducens Nerve Diseases ; Age Distribution ; Astrocytoma ; Brain Neoplasms* ; Brain* ; Central Nervous System ; Child* ; Choroid Plexus ; Craniopharyngioma ; Diagnosis ; Drug Therapy ; Ependymoma ; Female ; Follow-Up Studies ; Gait ; Glioma ; Headache ; Humans ; Infratentorial Neoplasms ; Male ; Medulloblastoma ; Neural Plate ; Neuroblastoma ; Papilledema ; Papilloma, Choroid Plexus ; Radiotherapy ; Reflex, Babinski ; Seizures ; Sex Ratio ; Skull ; Survival Rate ; Sutures ; Teratoma ; Tomography, X-Ray Computed ; Vomiting