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Neuroblastoma
Published in Mark Davenport, James D. Geiger, Nigel J. Hall, Steven S. Rothenberg, Operative Pediatric Surgery, 2020
Patients with neuroblastoma usually present with signs and symptoms that reflect the primary site and extent of disease, although localized disease can be asymptomatic. As 75% of neuroblastoma occurs in the abdominal cavity, an abdominal mass detected on physical examination is a common clinical feature, as is the complaint of abdominal pain. Other primary sites of neuroblastoma include the posterior mediastinum (20%), the cervical region (1%), and the pelvis (4%) (organ of Zuckerkandel) (Figure 60.1). Respiratory distress or dysphagia may reflect a thoracic tumor. Altered defecation or urination may be caused by mechanical compression from a pelvic tumor or by spinal cord compression from a paraspinal tumor. Spinal cord compression may also present as an altered gait. A tumor in the neck or upper thorax can produce Horner syndrome (ptosis, miosis, and anhydrosis), enophthalmos, and heterochromia of the iris. Acute cerebellar ataxia has also been observed, characterized by the dancing-eye syndrome, which includes opsoclonus, myoclonus, and chaotic nystagmus. Two-thirds of these cases occur in infants with mediastinal primary tumors. Additional signs and symptoms that reflect excessive catecholamine or vasoactive intestinal polypeptide (VIP) secretion include hypertension, diarrhea and weight loss.
Paper 1
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
The characteristics of the case in this question are suggestive of neuroblastoma; the patient is <2 years old and the mass is large, displacing the bowel and kidney and encasing the vasculature. On ultrasound it has areas which likely represent calcification. The mass extends adjacent to the vertebral column, and neural foraminal involvement is important to establish as it may impact management if neurosurgical input is required. Neuroblastoma frequently metastasises, commonly to the liver and bone. CT is frequently employed in the diagnosis and staging of neuroblastoma. To assess distant extent, MIBG has traditionally been the investigation of choice; however PET/CT has been used in some centres. Technetium-99m bone scans can also be helpful.
Pediatric Spinal Tumors
Published in David A. Walker, Giorgio Perilongo, Roger E. Taylor, Ian F. Pollack, Brain and Spinal Tumors of Childhood, 2020
Rajiv R. Iyer, Nir Shimony, Mohammad Hassan A. Noureldine, Eric Bouffet, George I. Jallo
Further studies have confirmed the benefit of this approach in patients with neuroblastoma.65,66 A series from Cairo on 51 neuroblastoma patients with clinical or radiological evidence of intraspinal extension included 34 patients with neurological deficits.67 Thirty of these 34 patients were treated with chemotherapy (16 patients) or chemotherapy concomitant with steroids (14 patients). Overall, 16 patients showed a complete recovery, 11 partial improvement, whereas 11 patients had no change in their neurological deficit. Improved outcome was significantly correlated with a shorter duration (<4 weeks) of symptoms.
Macrophage infiltration promotes regrowth in MYCN-amplified neuroblastoma after chemotherapy
Published in OncoImmunology, 2023
Anders Valind, Bronte Manouk Verhoeven, Jens Enoksson, Jenny Karlsson, Gustav Christensson, Adriana Mañas, Kristina Aaltonen, Caroline Jansson, Daniel Bexell, Ninib Baryawno, David Gisselsson, Catharina Hagerling
Neuroblastoma is a pediatric solid tumor that arises from the sympathetic nervous system and presents with a mass in the adrenal gland or along the sympathetic chain.1 It is the most common extracranial solid tumor in children accounting for 6–10% of pediatric cancers.2,3 Despite advances in risk stratification and therapy, neuroblastoma remains a therapeutic challenge, accounting for approximately 15% of all pediatric cancer deaths.4–6 Neuroblastoma is stratified into low-, intermediate- or high-risk groups, according to the International Neuroblastoma Risk Group (INRG) consensus criteria, which include age at diagnosis, histological category, and genetic characteristics such as MYCN amplification (MNA+).7 MNA+ is found in approximately 20% of all neuroblastoma patients and accounts for 40% of all high-risk neuroblastoma cases.8 Increased expression of MYCN is a tumor-initiating event responsible for the development of high-risk neuroblastoma.9,10 Besides having a direct effect on neuroblastoma development, MNA+ induces an immunosuppressive tumor microenvironment. MYCN negatively regulates ligands for natural killer (NK) receptors, and MNA+ is associated with the downregulation of MHC-I expression in neuroblastoma, rendering tumor cells less susceptible to recognition and killing by NK- and T-cells.11–13 Moreover, MNA-positivity correlates with the infiltration of tumor-associated macrophages (TAMs) into neuroblastoma tumors.13,14
Congenital Sacrococcygeal Neuroblastoma: A Report of Two Cases with Summary of Prior Published Cases
Published in Fetal and Pediatric Pathology, 2022
Soumya Dey, Arindam Ghosh, Kaushik Sil, Kalyani Saha Basu, Uttara Chatterjee
Case-2: A 6 days old baby boy presented with a swelling in the sacral region since birth. Serum AFP and hCG levels were within normal limits. MRI of lumbosacral spine showed a multilobulated mass below the distal end of spine and differential diagnosis of teratoma and terminal meningomyelocele were suggested (Fig. 3a and b). The mass was excised along with coccyx and on gross examination it was a bosselated, tan colored mass with patchy hemorrhagic and measured 5 cm. across (Fig. 3c). Microscopic examination revealed a tumor composed of small round cells with hyperchromatic nuclei and scanty cytoplasm arranged in nests. Extensive rosette formation and neuropil was identified in center of rosettes. The tumor had low MKI and minimal fibrillary stroma. On extensive sampling no heterologous elements were noted. Only remnant of coccyx was noted bearing notochordal elements in the center. On immunohistochemistry, the tumor cells were positive for synaptophysin and chromogranin (Fig. 4a–d). CD99, FLI-1, WT-1, TLE-1, desmin, myogenin, CD 117, OCT 4, LCA were negative. Based on the morphologic features and IHC findings, the diagnosis of poorly differentiated neuroblastoma was made. FISH analysis was negative for MYCN amplification. The baby was discharged on seventh post-operative day without any complications. On a fifteen months follow up, the patient is doing well without chemotherapy and urinary VMA level is normal. PET scan for metastatic workup was normal at 10 months of follow up.
Advances in pharmacotherapy for neuroblastoma
Published in Expert Opinion on Pharmacotherapy, 2021
Parmida Sadat Pezeshki, Aysan Moeinafshar, Faezeh Ghaemdoust, Sepideh Razi, Mahsa Keshavarz-Fathi, Nima Rezaei
Neuroblastoma is a heterogeneous pediatric tumor, that is mostly derived from the primitive sympathoadrenal lineage of neural crest cells [1]. It is the third most common pediatric cancer, and the most common cancer diagnosed in the first year of life, constituting 7% of cancer cases in this age group [2], and unfortunately about 15% of pediatric cancer death [3]. Besides its distinctive and diversified biology, neuroblastoma has a wide-ranging prognosis array: from spontaneous regression to aggressive metastatic tumors that are unresponsive to a variety of conventional and investigational therapies [4]. Neuroblastoma tumors most frequently develop in the medulla of the adrenal gland. In some cases, they can also originate from para-spinal sympathetic ganglia in the abdominal cavity, chest, or neck [5]. Studies show that the incidence rate in pediatric patients with neuroblastoma has been increased in the last decade, while the overall survival has been improved [6–8]. The incidence rate is marginally higher in boys than girls (8.5% vs 7.5%) [2]. No significant racial disparity has been observed in the incidence and survival rates of patients with neuroblastoma [8]. However, another study demonstrated that native Americans and African Americans have a higher risk of developing aggressive neuroblastoma tumors [9].