Primitive neuroectodermal tumor of the liver: a case report of a rare case in pediatrics

Montoya, Juan; Álvarez, Lina Marcela Cadavid; Gaviria, Jorge Alberto Ochoa; Cadavid, Juan Camilo Pérez

doi:10.1016/j.radcr.2021.05.001

Cited by 1 publication

(5 citation statements)

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“…They affect the paravertebral region, lower extremities, thorax, and abdomen. Abdominal involvement is rare and usually involves pancreas, stomach, and small intestine [ 5 ]. Peripheral primitive neuroectodermal tumors (pPNETs) account for 4-17% of all pediatric soft tissue tumors.…”

Section: Discussionmentioning

confidence: 99%

“…On her physical examination, she presented pale mucous membranes, multiple enlarged lymph nodes, and hepatomegaly. Total abdominal ultrasound showed hepatomegaly [ 5 ]. They did a chest CT of their patient, and it showed multiple subpleural, lung nodules compromising both lung fields.…”

Section: Discussionmentioning

confidence: 99%

“…Immunohistochemical staining was positive for chromogranin, CD99, neuron-specific enolase (NSE), synaptophysin, and FLI-1. chemotherapy was initiated [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

“…Only around 6% of them are extraosseous, but they typically start in the bones [ 4 ]. Clinically, PNETs present as abdominal pain, mass effect, and compression symptoms, such as abdominal distention and ascites [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

“…The radiologic findings of pPNET are not clear and make the diagnosis difficult [ 6 ]. A multimodal strategy comprising chemotherapy, radiation, and surgery has been reported for treatment and prognosis [ 5 ]. Here we focus on peripheral primitive neuroectodermal tumors (pPNETs) and report a rare case among the pediatric population.…”

Section: Introductionmentioning

confidence: 99%

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Peripheral Primitive Neuroectodermal Tumor: A Rare Case in Pediatrics

Rashed¹,

Alharthi²,

Aljabri³

et al. 2023

Cureus

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Primitive neuroectodermal tumors (PNETs) are a type of malignant tumors made up of small neuroectodermal-derived round cells that affect soft tissue and bone, with a wide range of clinical symptoms and histological commonalities depending on the site of the tumor. PNETs account for 4% of all pediatric and adolescent cancers. Here we report a case of a peripheral primitive neuroectodermal tumor in a five-year-old boy. Two days before admission, he complained of multiple attacks of vomiting and one episode of hematemesis, associated with subjective fever, abdominal pain, and distention. He also complained of weight loss and bruises on his face and lower extremities for the last four weeks. Upon physical examination, there was hepatomegaly to the right iliac fossa. Abdominal ultrasound showed that the liver is hugely enlarged with heterogeneous echo texture and smooth borders. A computed tomography scan with contrast showed hepatomegaly to the right iliac fossa region with no focal lesion. Bone marrow aspiration and bone marrow biopsy showed heavy infiltration by monomorphic cells. Moreover, liver biopsy was done for this patient, and it showed metastatic undifferentiated neuroblastoma. Before the liver biopsy results, the patient deteriorated rapidly and dead. Therefore, peripheral primitive neuroectodermal tumors (pPNETs) should be considered in the differential diagnosis of liver masses in young patients to early diagnosis and treatment, and to increase the survival rate.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Immunohistochemical staining was positive for chromogranin, CD99, neuron-specific enolase (NSE), synaptophysin, and FLI-1. chemotherapy was initiated [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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