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Histopathologic Prognostic Factors in Neuroblastic Tumors: Definition of Subtypes of Ganglioneuroblastoma and an Age-Linked Classification of Neuroblastomas
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1984
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Neuro-oncologyTumoral PathologyTumor InnervationMedicineHistopathologic Prognostic FactorsSurgical PathologyHistopathologyPretreatment TumorsPathologyNeuroblastsNeuroblastic TumorsNeurologyAge-linked ClassificationQuantitative MaturationNeuropathologyOncologyGliomaAggressive Behavior
The study classified 295 pretreatment neuroblastoma and ganglioneuroblastoma tumors into stroma‑poor and stroma‑rich groups, further subdividing them by age, maturation, MKI, and morphology to define prognostic subtypes with distinct survival rates. Patients with favorable stroma‑poor or well‑differentiated/intermixed stroma‑rich tumors had an 87% survival rate, whereas those with unfavorable stroma‑poor or nodular stroma‑rich tumors had only a 7% survival rate, reflecting aggressive morphology and high MKI.
Histopathologic prognostic factors of 295 pretreatment tumors of a total 641 neuroblastomas and ganglioneuroblastomas were studied with the use of the following proposed tumor classification. The tumors were divided into 2 groups: stroma-poor (235 cases) and stroma-rich (60 cases) according to their organizational pattern (stromal development). The stroma-poor group was classified further into 2 subgroups: favorable stroma-poor (84% survival) and unfavorable stroma-poor (4.5% survival) according to the patient's age at diagnosis, degree of maturation, and nuclear pathology [mitosis-karyorrhexis index (MKI)] of the neuroblastic cells. The stroma-rich group was further classified into 3 subgroups: well differentiated (100% survival), intermixed (92% survival), and nodular (18% survival) on the basis of morphology of the immature element in the tumor tissue without regard to patient's age or quantitative maturation. Favorable stroma-poor and well-differentiated and intermixed stroma-rich groups seem to make good prognosis groups (87% survival), which show gradual progression along a maturational sequence according to the age of the patient. Unfavorable stroma-poor and nodular stroma-rich groups form poor prognosis groups (7% survival) and show morphological evidence of malignant or aggressive behavior, such as inappropriate immaturity for age, higher MKI, and gross nodule formation by immature neuroblasts.