Primary malignant lymphoma of the central nervous system in an immunocompetent child: a case report

Primary lymphoma of the central nervous system (PCNSL) is extremely rare, especially in childhood. A 9-year-old Japanese boy was diagnosed as having precursor-B cell-type lymphoblastic lymphoma, based on morphologic and immunocytochemical analysis of mononuclear cells in the cerebrospinal fluid and a positive reaction for terminal deoxynucleotidyl transferase (TdT), CD19, CD79a, and CD179b. After seven courses of chemotherapy and craniospinal radiotherapy, the patient is alive, well, and in continuous complete remission. Despite its rarity, PCNSL should be included in the differential diagnosis in the presence of symptoms of increased intracranial pressure and/or unusual imaging findings of the brain.     

Childhood malignant lymphoma of bone

From 1974 to 1987, 450 children with non-Hodgkins' lymphoma (NHL) were seen at the Institut Gustave Roussy (IGR); 14 children had malignant lymphoma of bone (MLB). Eleven of the 14 were newly diagnosed, whereas three presented in relapse. Nine patients presented with multifocal bone involvement. The median age of these eight girls and six boys was 9.5 years (range 1.25-15 years). Bone pain was present in all patients as the initial symptom. Evaluation included physical examination, routine serum chemistries, complete blood count, chest roentgenography, skeletal survey, radionuclide bone scan, lumbar puncture, bone marrow aspiration, and intravenous pyelography, and/or abdominal ultrasonography. Hypercalcemia was found in six patients. Biopsy was performed in 12 patients, revealing high-grade lymphoblastic lymphomas in all. In two patients diagnosis was made on cytological examination of bone marrow aspirate. Immunophenotyping in four cases, demonstrated non-B, non-T cell origin in three and pre-B cell origin in one. Three patients were treated prior to 1982 with Cyclophosphamide/Oncovin/Prednisone/ADriamycin (COPAD) and seven patients, seen after 1982, were treated with a modified LSA2L2 protocol (LMT). None of the previously untreated patients received radiotherapy. All patients treated with COPAD have died, whereas four out of seven treated with LMT are alive with a median follow up of 51 months (range 36-82 months). One child treated on a pilot study died. One of the three children seen at relapse is disease-free with a follow-up of 98 months after high-dose chemotherapy followed by autologous bone marrow transplantation (ABMT). Five out of six patients presenting with hypercalcemia have died. Results with LMT are encouraging and together with published results suggest that sufficiently intensive chemotherapy can result in complete remission and cure in MLB. Radiotherapy does not seem to be necessary, avoiding possible serious long-term effects. Hypercalcemia is a bad prognostic feature.     

Primary malignant lymphoma cutis in an infant: case report and review of literature.

A 6-month-old infant with primary malignant cutaneous lymphoma is presented. The histologic differentiation between benign cutaneous lymphoid hyperplasia and cutaneous malignant lymphoma is discussed. Intensive therapy is warranted at the onset because of early dissemination and poor response to treatment after dissemination.     

Primary hepatic Burkitt lymphoma

Primary hepatic lymphomas are exceedingly rare in children, with less than a dozen cases described to date. The authors present an 8.5-year-old boy with Burkitt lymphoma of the liver who had isolated multifocal liver lesions that exhibited a multilayered progressive enhancing pattern on MRI. Diagnosis was achieved after laparotomy and incisional biopsy that failed to detect disease outside the liver. The patient received short, intensive multiagent chemotherapy. He is currently well 22 months after the end of chemotherapy. This case illustrates that clinical judgment in conjunction with unusual imaging findings may contribute to the diagnosis of common tumors in rare locations.     

Primary signet-ring-cell carcinoma of the rectum in a 12-year-old boy

A case of primary signet-ring-cell carcinoma of the rectum is presented. Colorectal carcinoma is uncommon in childhood; moreover, the signet-ring-cell type is very rare even in adults. The clinical, pathologic, and biologic characteristics of this carcinoma are different from ordinary colorectal carcinomas. Our patient was a 12-year-old boy, the youngest with a signet-ring-cell carcinoma of the rectum in the English literature, who had a Dukes' C lesion. Pathologically, the tumor is characterized by diffuse infiltration of the bowel wall by signet-ring-cells. The prognosis is extremely poor.     

Prostate cancer mimicking malignant lymphoma

We report three patients evaluated on a medical service for lymphoma-like signs and symptoms. Although none had prostate or bone symptomatology, all three were found to have metastatic prostate cancer. These cases emphasize the propensity of prostate cancer to metastasize to lymph nodes as well as bones. Diagnostic and therapeutic implications are discussed.     

Primary pineal malignant melanoma

Primary pineal malignant melanomas are a rare subset of primary central nervous system melanomas. This report presents the case of a 20-year-old female patient with a primary pineal region malignant melanoma who underwent endoscopic biopsy and adjuvant therapy. Her treatment consisted of stereotactic radiation to the pineal tumor, conventional whole-brain radiation and Temodar(R) for the disseminated disease. She required a ventriculo-peritoneal shunt for refractory ICP problems. This report details the clinical features of the case and summarizes the literature on a rare but aggressive neoplasm.     

Primary unilateral renal lymphoblastic lymphoma

A 4-year-old boy presented with a right-sided mass. A lobulated tumour in the right flank was felt on palpation, and radiological investigation revealed a solid, multinodular intrarenal tumour. On biopsy the tumour was found to be a lymphoblastic lymphoma. Accepted: 12 January 1996     

Primary posterior mediastinal Burkitt lymphoma

A 14-year-old boy admitted to our clinic with a 20-day history of fever, cough and respiratory distress. Mediastinal enlargement was observed on chest radiograph. Computed tomography of the chest demonstrated a large posterior mediastinal mass. The histopathological examination of the mass revealed Burkitt's lymphoma. We report this case because primary posterior mediastinal involvement in Burkitt lymphoma is extremely rare in childhood.     

Primary lymphoma of brain in childhood

Primary brain lymphoma is exceedingly rare during the first decade of life. We report an unusual case, not only being one of the youngest presented, but also because of the unusual CT features exhibited.     

Primary pleural lymphoma: an unusual presentation of childhood non-Hodgkin lymphoma

A case of primary pleural non-Hodgkin lymphoma in a 2.5-year-old girl is reported. The patient had pleural involvement as the initial and only manifestation of the disease. Histopathalogic examination showed lymphoblastic lymphoma of T-cell origin. The child received the modified LSA2-L2 protocol. During the maintenance treatment, she had an isolated central nervous system relapse and died of neutropenic sepsis. To the authors' knowledge this represents the first case report of primary pleural lymphoma in the childhood period.     

Primary subconjunctival lymphoma: an unusual presentation of childhood non-Hodgkin's lymphoma

Of 806 non-Hodgkin's lymphoma cases, only one primary subconjunctival lymphoma case was observed in Hacettepe Children's Hospital over a period of 16 years. The patient was a 12 year-old-boy with a subconjunctival mass on the upper part of his right eye. Biopsy revealed malignant lymphoma. Extensive investigations disclosed no other site of tumor. He was given the modified LSA2-L2 protocol. He has been in remission for 22 months. This may be the first report of a primary subconjunctival lymphoma in the childhood period.     

Primary B-cell lymphoma of the CNS in an infant

Primary B-cell lymphoma of the CNS is a rare tumor representing 0.2 to 2% of all primary CNS malignancies [1, 2]. The usual age of presentation is in the 4th to 6th decades. Sporadic cases have been reported in the pediatric population. However, even in this age group the tumor is extremely rare under the age of 5 [3, 4]. This is a report of a 3-year-old child with primary lymphoma of the CNS.