Fig 1.
Morphological and histological characterization of tumors from patient #1, presenting concomitant NB and ACT.
A) Schematic representation based on patient #1 image files demonstrating adrenocortical tumor in right adrenal gland and the neuroblastoma in left paravertebral region. B) Right adrenocortical tumor from patient #1 measuring 2,0 x 1,8 x 0,8 cm. The microscopic examination revealed in I) cortical region of non-tumoral adrenal; II) medullary region of non-tumoral adrenal; III) adrenocortical tumor characterized by atypical large pleomorphic cells with acidophilic cytoplasm, presenting Fuhrman nuclear atypia grade 3 (H&E 100x magnification). C) Histological examination of left paravertebral tumor from patient #1. The neuroblastoma presented dimensions of 4,3 x 3,2 x 1,5 cm and was characterized by small round primitive cell clusters (H&E staining, 40x magnification). In detail, neuroblasts at different maturation stages embedded in neurofibrillary stroma (H&E, 400x magnification).
Fig 2.
Morphological and histological characterization of tumors from patient #2, presenting concomitant NB and ACT.
A) Schematic representation based on patient #2 macroscopic findings shows the right adrenal gland presenting concomitant occurrence of adrenocortical adenoma and in situ neuroblastoma. B) The macroscopic examination of tumor demonstrates a solid and well-circumscribed adrenocortical tumor, completely surrounded by a thin fibrous capsule (arrow). The cut surface shows a yellowish, shiny, and homogeneous neoplasm with slightly lobular architecture. C) The left half of the picture shows the adrenocortical tumor surrounded by a thin and fibrous capsule (HE, 100x). Insert a detail demonstrating tumor cells of large size, with huge and eosinophilic cytoplasm, large nuclei, with central and conspicuous nucleoli. (HE, 400x). D) In situ neuroblastoma can be identified as a slightly pale region indicated by an arrow. E) Microscopic analysis from the pale region of remainder adrenal gland shows well-preserved cortical parenchyma cells (arrowheads). The inner medullary zone is heavily infiltrated by in situ neuroblastoma (arrows) (HE, 40x). Insert, a detail showing tumor composed of small round blue cells with scant cytoplasm, and dark, hyperchromatic nuclei with frequent formation of Homer-Wright rosettes (thin arrows) (HE, 100x).
Table 1.
Characteristics of p.R337H positive patients.
Fig 3.
Immunohistochemical p53 staining on p.R337H positive NB tumors.
A, B) Representative images of nuclear p53 accumulation on neuroblastoma cells from patients 3 and 7, respectively (100x magnification).
Table 2.
Clinico-biological data of neuroblastoma patients according to p.R337H status.
Table 3.
Cancer family history of the 83 neuroblastoma patients according to p.R337H status.
Table 4.
Literature review on TP53 polymorphisms in neuroblastoma.
Table 5.
Literature review on TP53 mutations in association with neuroblastoma.