|Year : 2013 | Volume
| Issue : 2 | Page : 308-310
Rare coexistence of metastatic neuroblastoma of liver and solid pseudo papillary tumor of pancreas: Case report and literature review
Jiao Zhang, Yinghua Liu, Pingqian Bao, Yi Wang, Yi Zhang
Department of Pediatric Surgery, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, China
|Date of Web Publication||13-Jun-2013|
Institute of Digestive Surgery, State Key Laboratory of Biotherapy, West China Hospital, Sichuan University, Chengdu, Sichuan 610041
Source of Support: None, Conflict of Interest: None
Neuroblastoma is the second most common malignant solid tumor in children, and often metastasizes to liver, most notably in patients with stage 4S tumors. Solid pseudopapillary tumor of the pancreas (SPT) is a pancreatic borderline tumor with low malignant potential. Coexistence of these two tumors in one patient has never been reported before. Hereby, we present a case of an 8-month-old infant with coexisting tumors of SPT and metastatic neuroblastoma of liver. Dysdifferentiation of neural crest might be responsible for histogenesis of the coexisting tumors.
Keywords: Metastatic, neural crest, neuroblastoma, solid pseudopapillary tumor of the pancreas
|How to cite this article:|
Zhang J, Liu Y, Bao P, Wang Y, Zhang Y. Rare coexistence of metastatic neuroblastoma of liver and solid pseudo papillary tumor of pancreas: Case report and literature review. J Can Res Ther 2013;9:308-10
|How to cite this URL:|
Zhang J, Liu Y, Bao P, Wang Y, Zhang Y. Rare coexistence of metastatic neuroblastoma of liver and solid pseudo papillary tumor of pancreas: Case report and literature review. J Can Res Ther [serial online] 2013 [cited 2020 Feb 27];9:308-10. Available from: http://www.cancerjournal.net/text.asp?2013/9/2/308/113402
| > Introduction|| |
Neuroblastoma is the second most common malignant solid tumor in children, and often metastasizes to liver, most notably in patients with stage 4S tumors. Solid pseudopapillary tumor of the pancreas (SPT) is a pancreatic borderline tumor with low malignant potential, mostly seen in young females and rarely in infants. In this study, we present a rare coexistence of SPT and metastatic neuroblastoma of liver. To our knowledge, the coexistence of these two tumors in one person has never been reported before.
| > Case Report|| |
An 8-month-old female infant with 1-month history of rapidly increasing right upper abdominal distension was admitted. She had no history of fever, jaundice, or gastrointestinal hemorrhage. Physical examination revealed a firm, palpable liver reachingthe the pelvic region. No abdominal mass or enlarged superficial lymph nodes were palpated. Serum vanilmandelic acid (VMA), alpha fetoprotein (AFP) and caicino-embryonic antigen (CEA) levels were normal. Contrast-enhanced computed tomography (CT) scan showed diffuse enlarged liver with numerous small low-density focuses, and adhesions occurred between liver, pancreas, and the posterior wall of stomach [Figure 1] and [Figure 2]. Head, chest, abdomen, and pelvis CT showed no other mass or lesion.
Exploratory laparotomy was performed. At the surgical staging, small amount of ascites was aspirated, and a purple, enlarged liver with diffuse solid and cystic nodules on the surface was detected. The body of pancreas was firm with no mass-like enlargement. The left liver lobe and pancreatic body were adhered tightly to the posterior wall of stomach. Explorations of other parts of the abdomen, including bilateral adrenal glands and kidneys, were negative. Part of the dense adhesions between the left liver and the surrounding tissues (about 5 × 5 × 4 cm in size) was excised, and biopsies were taken from the posterior wall of stomach.
|Figure 1: Coronal CT scan of the abdomen shows diffuse enlarged liver with numerous small low-density focuses|
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|Figure 2: Transverse CT scan of the abdomen shows pancreas adhered to the posterior wall of stomach with no mass-like enlargement. Insert shows normal bilateral adrenals and retroperitoneum|
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Hematoxylin and eosin (HE) staining of the liver tissues showed abundant tuberous metastases and clusters of small, round, blue cells with high nucleus-to-cytoplasm ratio and immature chromatin [Figure 3]. Immunohistochemical (IHC) analysis showed positivity for neuron-specific enolase (NSE)/chromogranin A (CgA), 20% positive rate of Ki67, and negativity for syn/AFP/CD99/desmin [Figure 4]. HE staining of the posterior wall of stomach tissues showed cystic degeneration and solid pseudopapillary formations with small to medium-sized, polygonal, monomorphous cells radially arranged around fibrovascular stalks, forming rosette-like patterns [Figure 5]. IHC analysis showed positivity for NSE/CD56/CD10/ β-catenin and negativity for syn/E-cadherin/insulin [Figure 6]. Technetium-99m methylene diphosphate (Tc 99m MDP) and iodine-123 metaiodobenzylguanidine (I 123 MIBG) were performed. Tc 99m MDP bone scan showed no abnormal foci of the whole body bones, and the I 123 MIBG scan showed no uptake of tracer activity. The final diagnosis was coexisting SPT and metastatic neuroblastoma of the liver with unknown primary site.
|Figure 3: HE staining of the liver tissues showing abundant tuberous metastases instead of normal lobular architecture (×100). Insert showing clusters of small, round, blue cells with high nucleus-tocytoplasm|
ratio and immature chromatin (×400)
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|Figure 4: Immunohistochemistry result of liver tissues: strongly positive for (a) NSE and (b) CgA; negative for (c) syn and (d) AFP (×400)|
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|Figure 5: HE staining of the posterior wall of stomach tissues showing cystic degeneration with solid and pseudopapillary formations (×100). Insert showing tumor cells radially arranged around fibrovascular stalks, forming rosette-like patterns (×400)|
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|Figure 6: Immunohistochemistry result of posterior wall of stomach tissues: strongly positive for (a) CD10 and (b) â-catenin; weakly positive for (c) NSE and (d) CD56 (×400)|
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OPEC regimen (vincristine, cisplatin, teniposide, and cyclophosphamide) was provided to the infant 2 weeks after the operation. Four courses of standard chemotherapy were provided. One-year follow-up indicated that the child's general condition was improved and liver metastases were reduced obviously with only small amount of residual tumors left. The tumor of SPT remained unchanged during the follow-up.
| > Discussion|| |
Neuroblastoma is a malignancy that arises in tissues of the sympathetic nervous system, typically in the adrenal medulla or paraspinal ganglia. More than 90% of the primary neuroblastomas could be detected by combined imaging examination, including CT and isotope.  But in this case, CT, I 123 MIBG scans, and operative exploration all showed no signs of primary tumor. The possible reasons are false negativity or the regression of the primary site. Most false negatives are mainly caused by tumor's intrinsic inability to take in I 123 MIBG or tumor's small size (usually <1 cm).  The combined application of multiple examination methods is effective to prevent false negatives. The neuroblastoma in this case is categorized to 4S stage according to the International Neuroblastoma Staging System (INSS). An impressive regression is also the specialty of stage 4S neuroblastoma which mostly demonstrated as favorable biologic features and relentless early progression and ultimately full and complete regression of the primary tumor. 
SPT is a low-malignant pancreatic tumor. Its clinical presentation is usually nonspecific, and diagnosis mainly depends on diverse immunophenotypes and characteristic histopathologic features. As a rare coexisting phenomenon of neuroblastoma and SPT, the origin of these two tumors might have some kind of correlation.
It has already been clarified that neuroblastoma originates from the embryonic neural crest, while the histogenesis of SPT is still undefined owing to the diversity of immunophenotype. Some studies indicated an origin from ductal epithelium, acinar cells, or genital ridge/ovarian anlage-related cells. ,, But some other investigators favor the theory that SPT originates from pancreatic pluripotential stem cells based on immunohistochemical staining for vimentin, α1-antitrypsin, and NSE. , However, pluripotential stem cells isolated from pancreas were verified to have positive expression of nestin, a neural crest marker.  So, it is possible that neural crest plays an important role in the histogenesis of SPT. Another proof is that the formation of premelanosomes and melanosome granules was found in one patient's SPT cells.  Since melanocyte was demonstrated to derive from neural crest, this finding of melanocytic differentiation implied that cells of SPT might derive from neural crest. Conclusively, neuroblastoma and SPT might have the same origin, and dysdifferentiation of neural crest might lead to the concurrence.
In conclusion, this is a rare coexistence of SPT and metastatic neuroblastoma of liver with unknown primary site, and dysdifferentiation of neural crest might be responsible for the histogenesis of the coexisting tumors.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]