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The histological features of primary and metastatic neuroblastic tumors in a representative case. (A) Primary tumor initially diagnosed as neuroblastoma (Schwannian stroma-poor), poorly differentiated subtype. (B) Primary tumor after multiple cycles of chemotherapy showing maturation evidenced by scattered differentiating neuroblasts in Schwannian stroma and fibrosis. (C) Metastatic tumor in bone marrow at initial diagnosis composed of undifferentiated neuroblasts in neuropil. (D) Metastatic tumors in bone marrow after chemotherapy showing differentiating neuroblasts or ganglion cells in Schwannian stroma.
Source publication
The aim of this study is to evaluate the histologic features of metastatic neuroblastic tumors (NTs) in bone marrow (BM) before and after chemotherapy in comparison with those of primary NTs.
A total of 294 biopsies from 48 children diagnosed with NTs with BM metastasis were examined. There were 48 primary neoplasm biopsies, 48 BM biopsies before c...
Contexts in source publication
Context 1
... taken from both the right and left pelvic bones. Metastatic NTs in bone marrow were evaluated by histological review of H&E and IHC sections. Metastatic tumors consisted mainly of undifferentiated and/or differentiating neuroblasts. Neuropil was observed in 43 patients (89.6%). Neither gangli- on cells nor Schwannian stroma was detected (Table 2, Fig. 2C). We categorized histological subtypes of metastatic NTs in bone marrow and compared these findings to their original primary tumor subtype (Fig. 1). Regardless of the primary tumor sub- type, the most common subtype of metastatic NT in bone mar- row was the poorly differentiated subtype (n=38, 79%). How- ever, cases with more ...Context 2
... of primary neoplasms were performed in 36 patients after multiple cycles of chemotherapy. Histology before and after chemotherapy was compared in 33 cases exclud- ing three cases in which histologic subtyping before chemother- apy was not feasible due to limited tissue. After chemotherapy, 17 cases showed histological differentiation (52.4%) (Fig. 2B). Poorly differentiated and undifferentiated NB subtypes at ini- tial diagnosis showed frequent differentiation (82.4%). Fourteen cases were found to have the same histological grade regardless of chemotherapy (43.4%). Only one case was found to have a more immature subtype after chemotherapy, which morphed from differentiating NB into ...Context 3
... cycles of chemotherapy, repeat bone marrow biopsies were performed, and 23 of 48 cases had persistent met- astatic NTs in the bone marrow (47.9%). These metastatic sites showed a variable degree of differentiation after chemotherapy. Ganglion cells and Schwannian stroma were found in addition to undifferentiated and differentiating neuroblasts (Figs. 2D, 3C). Other histological features in the bone marrow included decreased neuropil and increased necrosis following chemother- apy. Persistent metastatic NTs in subsequent bone marrow bi- Regardless of the primary tumor subtype, poorly differentiated or differentiating subtypes predominated at the time of diagnosis. NBU, neuroblastoma ...Similar publications
Objective:
Intracerebral metastases in neuroblastoma patients are rare, and information about the indication for and the outcome of neurosurgical procedures in this setting is scarce in the literature. The authors' aim in the present study was to report a single-center experience with the neurosurgical treatment of intracerebral metastases in neur...
Citations
Background:
Although survival rate among patients with non-high-risk neuroblastoma is excellent, a gross residual tumor (GRT) is often present at the end of treatment. However, reliable data do not exist on the relevance of a GRT for the risk of progression and the role of adjuvant therapy for patients with GRT.
Methods:
A retrospective review of 131 patients with non-high-risk neuroblastoma who underwent chemotherapy was performed. GRT was defined as >1 cm3 residual soft tissue density on end-of-chemotherapy scans. Progression-free survival (PFS) and overall survival (OS) rates were compared between patients with GRT and those without GRT. A proportional hazards model was also used to assess the effects of GRT and adjuvant therapies, including radiation and isotretinoin therapy on outcomes.
Results:
GRT was found in 52 (40%) patients in the study cohort. Correlation was not found between GRT and outcomes (PFS; p = .954, OS; p = .222). In multivariable analysis, GRT remained a nonsignificant predictor of outcome after adjusting for confounders. Local radiation and isotretinoin therapy did not affect outcome for patients with GRT. However, within GRT subgroups, the degree of volume reduction, as well as absolute residual volume in the primary tumor after induction treatment, were significantly associated with outcomes.
Conclusion:
GRT in non-high-risk neuroblastoma may not indicate active disease that requires additional treatment. However, risk of progression is increased in patients with GRT whose response to treatment was less prominent, thus adjuvant therapy should be reserved only for those patients.
Neuroblastoma is the most common extra-cranial solid tumour in children. Bone marrow examination is a part of diagnosis and staging workup of neuroblastoma. Chemotherapy forms mainstay of treatment and post-chemotherapy histological changes are an important indicator of prognosis. A 4-year-old male child came with complaints of fever and hepatomegaly. Investigations revealed a retroperitoneal neuroblastoma. Bone marrow was involved by neuroblasts (stage 4S). Platinum-based chemotherapy was started and post-induction phase bone marrow showed differentiation of neuroblasts to ganglion cells and schwannian stroma (tumour load <5%, minimal disease). The patient was lost to follow up thereafter. The patient returned after one year with the reappearance of neuroblasts (tumour load >20%, relapse) in the bone marrow. Use of immunohistochemical markers like chromogranin and S100 are helpful to map the tumour load and identify the tumour cells when they are sparse. Persistence / reappearance of neuroblasts post-chemotherapy or increasing tumour load indicate a relapse.
