Neuroendocrine Tumor Arising within Mature Cystic Teratoma of the Pancreas: Literature Review and Case Report

Djokić, Mihajlo; Hadzialjevic, Benjamin; Ranković, Branislava; Dežman, Rok; Tomažič, Aleš

doi:10.3390/curroncol29070374

Cited by 2 publications

(3 citation statements)

References 29 publications

(32 reference statements)

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“…7 Primary retroperitoneal nets are exceedingly rare with only 8 previously reported cases 8 and only 3 prior reports of cystic teratomas containing NET components. 9 We present a rare case his 1-year post-operative visit, he reported occasional facial flushing and diarrhea although his 5-HIAA remained within the normal range, as well as anejaculation. Imaging was negative for disease recurrence.…”

Section: Introductionmentioning

confidence: 82%

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Case – Giant primary retroperitoneal teratoma with neuroendocrine components

Sellke,

Tay,

Zhou

et al. 2023

CUAJ

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of a 27-year-old man with a 38 cm primary retroperitoneal teratoma (RT) with well differentiated NET and presence of erectile-type tissue. CASE REPORTA 27-year-old male presented with COVID-19 related symptoms. Chest imaging obtained during work up revealed a retroperitoneal mass. He reported an increase in abdominal girth, but denied pain, gastrointestinal symptoms, or weight change. Past medical history and family history were unremarkable. On exam, he had a large, firm, palpable left sided abdominal mass. Scrotal exam was unremarkable with no palpable testicular masses. Preoperative tumor markers were not obtained. On CT, the mass was 30 cm in greatest dimension with solid and cystic areas, and located in the retroperitoneum, anterior to the left kidney and displacing the left ureter anteriorly.(Figure 1). The tumor was believed to be a sarcoma prior to resection.The patient subsequently underwent an open, transperitoneal excision of the retroperitoneal tumor. A midline incision extending from his xiphoid to his pubis was utilized. Despite the tumor's proximity to the spleen and inferior mesenteric artery, all structures were able to be dissected free and spared. With the help of a ureteral stent, the left ureter was identified anterior to the mass and was also dissected free without injury. Parasitic vessels arose directly from the aorta and implanted into the medial tumor. These were effectively ligated with bipolar diathermy (Figure 2). Recovery was uneventful and he was discharged on postoperative day 3.Gross examination of the excised tumor revealed a multilocular solid and cystic mass, 38 cm in largest dimension, primarily comprised of yellow adipose tissue with cystic areas containing thick, yellow grumous brown-tan keratinous material. Hair, bone, and cartilage were also identified. Histologic examination revealed tissue derived from all three germ layers with epidermal (squamous and columnar epithelium lined cysts), mesodermal (adipose, cartilage, bone, and fibrous tissue), and endodermal elements (gut epithelium). There was notably vascular tissue resembling erectile tissue identified. Two foci incidentally identified on histology were comprised of well-differentiated neuroendocrine cells that showed diffuse immunocytochemical expression of synaptophysin, chromogranin, SATB2, and CDX2 without pancreatic polypeptide, glucagon, or somatostatin, a pattern of staining resembling NET of distal small bowel/appendiceal NET. The Ki-67 proliferation index demonstrated nuclear staining in 2% of cells (Figure 3). There was no gross or histologic evidence of a background supernumerary testis. These findings along with the imaging findings are consistent with well differentiated enterochromaffin cell NET, grade 1 arising in a primary retroperitoneal mature cystic teratoma.Postoperative tumor markers were within normal limits (alpha fetoprotein [AFP], betahuman chorionic gonadotropin [bhcg]). Post-operative scrotal ultrasound was negative. Plasma 5-HIAA was obtained due to NET finding and within normal limits. Tum...

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Section: Introductionmentioning

confidence: 82%

“…Primary mature RT with concomitant NET is rare with only three prior reported cases. 9 Carcinoid syndrome is not a feature of retroperitoneal nets 11 and mass effect is not detected until the tumor is very large. 12 Prior cases report tumor size ranging from 3.5cm to 21cm 8 making ours the largest at 38 cm.…”

Section: Discussionmentioning

confidence: 99%

Case – Giant primary retroperitoneal teratoma with neuroendocrine components

Sellke,

Tay,

Zhou

et al. 2023

CUAJ

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“…[1] According to carcinoid tumor patients registered from 1950 to 1999 in the Surveillance, Epidemiology, and End Results database, NETs in the gastrointestinal tract and bronchopulmonary system accounted for 66.9% and 24.5% of all sites, respectively. [2] In addition, the primary sites of NETs vary in sex and race. Specifically, the common sites of NETs in female patients are the lungs, stomach, appendix, and cecum, whereas, in male patients, they are the thymus, duodenum, pancreas, jejunoileum, and rectum.…”

Section: Introductionmentioning

confidence: 99%

Primary intraspinal neuroendocrine tumor: A case report and literature review

Dai,

Zou,

Liao

et al. 2024

Medicine

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Rationale: Neuroendocrine tumors (NET) refer to a group of uncommon tumors arising in the neuroendocrine system. Most NETs occur in the digestive tract and bronchi but are rare in the central nervous system, especially in the spinal canal. NET in the central nervous system mainly metastasize from other systems, with non-specific clinical symptoms. In this study, we report the diagnosis and treatment of intraspinal NET to provide clinical guidance as well as to avoid misdiagnosis and missed diagnosis. Patient concerns: A 59-year-old male patient, presented with recurrent right lower limb pain for half a year, accompanied by numbness and weakness for 4 months and aggravation for 2 months. Lumbar spine magnetic resonance imaging (MRI) revealed a space-occupying lesion in the spinal canal. The diagnosis of primary intraspinal NET was confirmed by topathological examination. Diagnosis: Primary intraspinal NET tumor. Interventions: Surgical resection. Outcomes: Significant improvements in right lower limb pain, numbness, and weakness were observed, and lumbar spine MRI was performed again to dynamically observe the changes in intraspinal NET. Conclusions: Surgical resection may be an effective treatment for intraspinal NETs. Lessons: Intraspinal NETs are relatively rare and mostly manifest as limb numbness, weakness, and pain. Due to its nonspecific clinical symptoms, intraspinal NETs are easily misdiagnosed as lumbar disc herniation with radiculopathy and lumbar spondylolisthesis. Therefore, in patients with long-term symptoms, in addition to common lumbar neuromuscular diseases, lumbar MRI should be performed promptly to exclude the possibility of lumbar NETs.

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Neuroendocrine Tumor Arising within Mature Cystic Teratoma of the Pancreas: Literature Review and Case Report

Cited by 2 publications

References 29 publications

Case – Giant primary retroperitoneal teratoma with neuroendocrine components

Case – Giant primary retroperitoneal teratoma with neuroendocrine components

Primary intraspinal neuroendocrine tumor: A case report and literature review

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