Management of tumor rupture and abdominal compartment syndrome in an infant with bilateral high risk stage 4 neuroblastoma

Abstract

Rationale: Tumor rupture and bleeding at initial presentation of infants with neuroblastoma (NBL) is a rare, but life threatening condition and challenge in pediatric oncology. Here, we report successful multidisciplinary management of an abdominal compartment syndrome as a result of tumor rupture and bleeding in an infant with bilateral high risk stage 4 NBL. Patient concerns: The patient was admitted to a cooperating hospital with vomiting, failure to thrive and a large mass in the abdomen and was then referred to our center. Diagnoses: Stage 4 NBL with MYC-N amplification and 1p36 deletion was diagnosed in an 11 months old girl. Due to rapid and massive tumor growth she developed abdominal compression with renal failure, severe bleeding, and tumor lysis syndrome (TLS). Interventions: Surgical decompression by enterostomy, local, and systemic bleeding control with platelets and coagulation factors, antiinfective and TLS therapy were effective in stabilizing the patient's condition. This allowed initiation of the multimodal antineoplastic treatment according to protocol NB 2004. Outcomes: Mechanical ventilation was stopped after 11 days, the abdominal wall was closed 3 months after the start of therapy, and treatment according to the protocol be started and successfully completed. Lessons: Only the immediate, coordinated multidisciplinary intervention managed to overcome the life-threatening abdominal compartment syndrome and its associated problems, eventually enabling successful curative treatment.

Hemorrhage During Induction Chemotherapy in Neuroblastoma: Additional Risk Factors in High-Risk Patients

“…Patients in group A had significantly higher LDH levels at diagnosis compared to group B; such data are consistent with the work by Qin et al and other published case reports ( 8 , 9 , 17 ). High serum LDH levels at diagnosis are associated with poorer outcome in terms of event-free and overall survival in high-risk neuroblastoma ( 25 ) and can be interpreted as the serum marker of high tumor burden.…”

“…The mechanism underlying spontaneous hemorrhage in neuroblastoma has been debated; in neonates, an adrenal mass could be crushed between the liver and the spine during delivery, causing tumor rupture and subsequent hemorrhage (14), while in older children the presence of neuroblastoma could predispose to adrenal hemorrhage following minor trauma (15). Most authors report massive hemorrhage in patients younger than 18 months affected by high risk neuroblastoma with N-MYC amplification (7,8,13,(15)(16)(17). In their large case series, Qin et al found two independent risk factors on multivariate analysis, i.e., the presence of N-MYC amplification and high tumor bulk, measured as maximum diameter of the primary mass.…”

“…Most authors report massive hemorrhage in patients younger than 18 months affected by high risk neuroblastoma with N-MYC amplification ( 7 , 8 , 13 , 15 – 17 ). In their large case series, Qin et al found two independent risk factors on multivariate analysis, i.e., the presence of N-MYC amplification and high tumor bulk, measured as maximum diameter of the primary mass.…”

A Case of Lethal Abdominal Compartment Syndrome due to Rapidly Expanding Ovarian Small Cell Carcinoma Pulmonary Type

“…Rapidly expanding intra-abdominal malignancies are an uncommon cause of ACS, and there are only a few cases of such in medical literature. In children, these malignancies have included neuroblastomas, nephroblastomas, and immature teratomas [8,9]. In adults, these primary tumors are often gynecological in nature, primarily benign ovarian cystadenomas with additional case reports of an ovarian granulosa cell tumor and a Burkitt lymphoma of the small intestine [10][11][12][13][14][15].…”

Akutes Abdomen im Kindesalter

“…Ein weiteres Beispiel eines chirurgisch-onkologischen Notfalls ist ein akutes Abdomen aufgrund eines Kompartmentsyndroms durch einen raumfordernden soliden abdominalen Tumor. Zum Beispiel kann ein Neuroblastom im Säuglingsalter mit Hepatomegalie durch Filiarisierung in die Leber eine Abdominozenthese zur Entlastung des abdominalen Drucks notwendig machen [ 9 ]. Grundsätzlich gilt, dass bei einem Kind mit distendiertem Abdomen immer an die Differenzialdiagnose einer abdominalen Raumforderung gedacht werden sollte.…”