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Two synchronous neonatal tumors. (a) Newborn with facial and cervical mass. (b) Magnetic resonance imaging showing cervical multinodular mass and liver tumor. (c) Light microscopy shows sialoblastoma with nests of basaloid (epithelial) cells and a cribriform pattern (H&E, 20x HPF). (d) The proliferative index revealed an increased number of nuclei staining positively with Ki67 (clon Mib-1, immunohistochemistry, 40x HPF).
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We report a case of a newborn with two synchronous tumors—sialoblastoma and hepatoblastoma—diagnosed at 20 weeks of gestation by magnetic resonance imaging (MRI) and ultrasonography (US). The aim of this study was to describe the management of this case together with a review of the literature. Our patient had a large facial tumor associated with e...
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Citations
... 62 In this systematic review, only 5 (8.0%) cases of sialoblastoma were diagnosed in the intrauterine period. 19,20,25,45,52 A total of T A B L E 2 Histological characteristics of 62 sialoblastomas included in this systematic review. ...
... 36 There are four reports in the literature demonstrating the synchronic association of sialoblastoma and hepatoblastoma, which is an extremely uncommon event. 20,34,50,52 Such occurrence can be explained due to the common embryonic origin of the parotid gland and the liver. As a result, the abnormality would affect the cells of both organs, leading to the appearance of two synchronous tumor producers of alpha-fetoprotein (AFP) in different sites in the same patient. ...
Sialoblastoma is a rare malignant salivary gland tumor. The aim of this study was to review the available published data on sialoblastoma in a comprehensive analysis of its clinicopathologic characteristics, treatment, and outcomes. An unrestricted electronic search was performed in the following databases: MEDLINE/PubMed, EMBASE, Scopus, Web of science, and gray literature databases. Eligibility criteria included publications with sufficient clinical, imaging, and histopathological information to confirm the diagnosis of sialoblastoma. Data were evaluated descriptively and analytically. A total of 52 studies met the eligibility criteria. In total, 62 patients were evaluated. There was no gender predilection, with the parotid being the most affected primary site (n = 28; 45.2%). In the log-rank test, there was a significant increase in disease-associated survival in patients younger than 1 year of age (82.8% vs. 44.4%; p = 0.003), individuals with lesions in major salivary glands (79.4% vs. 38.5%; p = 0.005), patients without metastases (77.8% vs. 14.3%; p = 0.011), encapsulated lesions (85.7% vs. 0%; p < 0.0001), congenital lesions (83.3% vs. 25.0%; p < 0.0001), and lesions that do not show perineural invasion (89.5% vs. 40%; p = 0.035). Kaplan-Meier curves estimated overall survival and disease-free survival at 5 years of 95.5% and 68.1%, respectively. In the multivariate Cox regression model, only the presence of metastasis was identified as an independent prognostic factor (hazard ratio [HR] = 9.81; p = 0.010). Although sialoblastoma presents good prognosis, the tumor has a high recurrence rate.
... A minimum of 20 cases described to date were treated with chemotherapy. Most of those cases responded well to the treatment, suggesting that sialoblastoma is a chemosensitive tumor [5,7,8]. Some of the older patients also received radiotherapy, which resulted in a good response [9,10]. ...
Sialoblastoma is an extremely rare embryonal tumor derived from salivary gland primordial cells. Treatment usually consists of surgery alone; however, in some cases, chemotherapy is required and is administered with good response. We present a case of a 5-week-old girl diagnosed with a parotid gland tumor and co-existing nevus sebaceous on the face. Initial tumorectomy was microscopically non-radical and histopathology revealed sialoblastoma. The patient received adjuvant chemotherapy consisting of vincristine, actinomycin, and cyclophosphamide. Due to imaging studies being inconclusive regarding response and possible residual disease, a second surgery (total parotidectomy) was performed. The histopathology results showed fields of necrosis in the parotid gland but no neoplastic cells in the material. The patient remains under watchful observation and there is no evidence of relapse 12 months after the second surgery. The adjuvant chemotherapy regimen with vincristine, actinomycin, and cyclophosphamide is a viable option of treatment in children with sialoblastoma.
... The majority of these patients were also diagnosed with Fanconi Anemia (FA), an autosomal recessive inherited syndrome characterized by congenital defects, aplastic anemia, and a high likelihood for cancer (10,11). As for simultaneous SBL and HB, only 5 cases have been previously reported without in-depth investigation of potential mechanisms (12)(13)(14)(15)(16). Underlying molecular and cellular features of simultaneous SBL and HB were poorly understood. ...
... As tumors from different organs, the synchronous occurrence of SBL and HB has rarely been revealed, with only 5 previously reported cases ( Table 3) (12)(13)(14)(15)(16). After summarizing all cases, we found that almost all patients were prenatally diagnosed with well-differentiated histology, further revealing the embryonal origin of both tumors and providing clues into the development similarities of salivary gland and liver. ...
Sialoblastoma (SBL) is an infrequent embryonal malignant tumor originating from the salivary gland, resembling primitive salivary gland anlage, whereas hepatoblastoma (HB) is the most common pediatric liver malignancy. The simultaneous occurrence of both tumors is extremely rare. Here we reported a case of a 6-month-old infant diagnosed with synchronous SBL and HB. The patient received neoadjuvant chemotherapy followed by surgical resection. Fresh tissues of both tumors were collected before and after chemotherapy, which were further profiled by whole exome sequencing (WES) and single-cell RNA sequencing (scRNA-seq). WES analysis revealed potential somatic driver mutation PIK3CA p.Glu454Lys for SBL and canonical mutation CTNNB1 p.Ser45Pro for HB. No shared somatic variants or common copy number alterations were found between SBL and HB primary tumor samples. Though scRNA-seq, single-cell atlases were constructed for both tumors. SBL may recapitulate a pre-acinar stage in the development of salivary gland, including basaloid, duct-like, myoepithelial-like, and cycling phenotypes. In the meantime, HB was composed of tumor cells resembling different stages of the liver, including hepatocyte-like, hepatic progenitor-like, and hepatoblast-like cells. After chemotherapy, both tumors were induced into a more mature phenotype. In terms of transcriptional signatures, SBL and HB showed enhanced expression of epithelial markers KRT8, KRT18, and essential embryo development genes SDC1, MDK, indicating the disruption of normal embryo epithelium development. Finally, heterozygous deleterious germline mutation BLM and FANCI were identified which could predispose the patient to higher cancer risk. It partially explained the reason for the co-occurrence of SBL and HB. Taken together, we provided valuable resources for deciphering cellular heterogeneity and adaptive change of tumor cells after chemotherapy for synchronous SBL and HB, providing insights into the mechanisms leading to synchronous pediatric tumors.
