HLA-mismatched family members may represent an important cell source for patients that require stem cell transplantation but lack both a matched sibling donor and a closely matched unrelated donor. We report the outcome of 19 transplantations from HLA two-or three-loci mismatched parental donors in which 14 pediatric patients with hematological malignancies or other disorders, received a median of 21.5 ؋ 10 6 (range, 5.4-58) highly purified CD34؉ peripheral blood stem cells (
BACKGROUND Mucosa‐associated lymphoid tissue (MALT) lymphoma is a distinct entity with specific clinical and pathologic features that may affect diverse organs. MALT–lymphomas remain localized within their original environment for a long period of time. As recent data have demonstrated a relatively high rate of multiorgan involvement at diagnosis, the authors have retrospectively evaluated 36 patients presenting with MALT‐lymphoma in the head and neck area. The authors focused on patients' disease localization, initial treatment, clinical course, and follow‐up. METHODS Thirty‐six patients with a histologically verified diagnosis of an extranodal marginal zone B‐cell MALT‐lymphoma arising in the head and neck area were included in this retrospective analysis. RESULTS Treatment consisted of surgical resection as the sole treatment in 4 patients (11%), surgical resection with consecutive radiotherapy in 13 patients (36%), radiotherapy alone in 11 patients (31%), chemotherapy in 2 patients (6%), surgical resection plus radiotherapy and chemotherapy in 4 patients (11%), and combined radiation and chemotherapy in 1 patient (3%). Complete and partial disease remissions after initial treatment were achieved in 22 (61%) and 13 patients (36%), respectively, whereas one patient refused any therapy. Four patients (11%) were lost to follow‐up and 15 patients (43%) have had disease recurrence after a median time of 11 months (range, 3–80 months). CONCLUSIONS These data suggest that MALT‐lymphomas of the head and neck area are preferentially treated using local modalities such as radiation and/or resection. This practice, however, is associated with an unexpectedly high rate of dissemination or disease recurrence. Obtaining an initial complete response is crucial in these patients. According to previous data, the possibility of understaging in such patients cannot be ruled out. Clinical trials with application of systemic treatment are warranted for these patients. Cancer 2003;97:2236–41. © 2003 American Cancer Society. DOI 10.1002/cncr.11317
Background/Objectives: The practice of palliative radiation therapy (RT) is based on extrapolation from adult literature. We evaluated patterns of pediatric palliative RT to describe regimens used to identify opportunity for future pediatric-specific clinical trials. Results: Of 3,225 pediatric patients, 365 (11%) were treated with palliative intent to a total of 427 disease sites. Anesthesia was required in 10% of patients. Treatment was delivered to metastatic disease in 54% of patients. Histologies included neuroblastoma (30%), osteosarcoma (18%), leukemia/lymphoma (12%), rhabdomyosarcoma (12%), medulloblastoma/ependymoma (12%), Ewing sarcoma (8%), and other (8%). Indications included pain (43%), intracranial symptoms (23%), respiratory compromise (14%), cord compression (8%), and abdominal distention (6%).Sites included nonspine bone (35%), brain (16% primary tumors, 6% metastases), abdomen/pelvis (15%), spine (12%), head/neck (9%), and lung/mediastinum (5%). Re-irradiation comprised 16% of cases. Techniques employed three-dimensional conformal RT (41%), intensity-modulated RT (23%), conventional RT (26%), stereotactic body RT (6%), protons (1%), electrons (1%), and other (2%). The most common physician-reported barrier to consideration of palliative RT was the concern about treatment toxicity (83%). Conclusion:There is significant diversity of practice in pediatric palliative RT. Combined with ongoing research characterizing treatment response and toxicity, these data will inform the design of forthcoming clinical trials to establish effective regimens and minimize treatment toxicity for this patient population. K E Y W O R D Spalliative radiation therapy, palliative therapy, pediatric radiation therapy Abbreviations: RT, radiation therapy; SBRT, stereotactic body radiation therapy
Mucosa associated lymphoid tissue (MALT) developing in response to chronic infection or autoimmune stimuli has been recognized as a possible site of origin for a distinct type of B-cell lymphoma. While preferentially occurring in the stomach, MALT-type lymphomas can be found in virtually all organs. MALT-type lymphomas normally follow an indolent course, with a tendency to remain localized at their site of origin for a prolonged period of time. Histologically, MALT-type lymphomas are heterogeneous covering a cytological spectrum ranging from centrocyte-like cells to smaller lymphoid cells or monocytoid B-cells. Usually a small number of transformed blasts are also present. Immunohistochemically, the malignant cells express markers of B-cell lineage, but are distinct from follicular lymphomas (which express CD10), mantle cell lymphomas (expressing cyclin D1 and CD5) and small lymphocytic lymphoma, which express CD5 and CD23. In contrast to the usual phenotype CD20+CD10-CD5-Cyclin D1-, scattered reports in the literature have documented expression of CD5 in marginal zone B-cell lymphomas of MALT-type. However, these cases are rare, and aberrant CD5-expression has been thought to be a marker for early dissemination and aggressive behavior in some patients, while other reports have also found CD5 expression in localized indolent MALT-type lymphomas. We report a patient with a CD5+ MALT-type lymphoma following an aggressive clinical course without histological progression who relapsed only 18 months after local radiotherapy at the initial localizations (conjunctiva of the right upper eye lid and hypopharynx), and showed a rapid generalization to the contralateral conjunctiva, mediastinal lymph nodes and the esophagogastric junction. Our case lends further support to the notion that CD5+ MALT-lymphomas arising in the head-and-neck area and/or the ocular adnexa might be characterised by an aggressive clinical course.
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