Adenocarcinoma and neuroendocrine tumor arising within presacral teratoma associated with Currarino syndrome: A case report

Chatani, Shohei; Onaya, Hiroaki; Kato, Seiichi; Inaba, Yoshitaka

doi:10.4103/ijri.ijri_148_19

Cited by 4 publications

(2 citation statements)

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“…In the literature, an association of presacral NENs with tailgut cysts and teratomas has been described. Additionally, an association with Currarino syndrome can be observed, an autosomal-dominant disorder caused by mutations in the motor neuron and pancreas homeobox 1 (MNX1) gene and characterized by presacral mass, sacral dysgenesis, anorectal anomalies (21,49,53). Most remarkably, whereas tailgut cysts or at least partially cystic primary tumors were observed in some of our patients, only one association with teratoma and none of other abnormalities like anorectal malformations or Currarino syndrome were present.…”

Section: Discussionmentioning

confidence: 63%

Multicenter Analysis of Presacral Neuroendocrine Neoplasms—Clinicopathological Characterization and Treatment Outcomes of a Rare Disease

et al. 2021

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Background and AimsNeuroendocrine neoplasms (NENs) of the presacral space are an extremely rare disease entity with largely unknown outcome and no established standard of care treatment. Therefore, we wanted to analyze clinical presentation, histopathological findings, treatment outcomes, and prognosis in a multicentric patient cohort.MethodsWe searched local databases of six German NEN centers for patients with presacral NEN. Retrospective descriptive analyses of age, sex, stage at diagnosis, symptoms, grade, immunohistochemical investigations, biomarkers, treatment, and treatment outcome were performed. Kaplan–Meier analysis was used to determine median overall survival.ResultsWe identified 17 patients (11 female, 6 male) with a median age of 50 years (range, 35–66) at diagnosis. Twelve cases presented initially with distant metastases including bone metastases in nine cases. On pathological review the majority of patients had well-differentiated G2 tumors. Immunohistochemical profile resembled rectal NENs. All but one patient had non-functioning tumors. Somatostatin receptor imaging was positive in 14 of 15 investigated cases. Eight patients were treated surgically including palliative resections; 14 patients received somatostatin analogs with limited efficacy. With 14 PRRTs completed, 79% showed clinical benefit, whereas only one patient with neuroendocrine carcinoma (NEC) responded to chemotherapy. Treatment with everolimus in three patients was not successful, whereas cabozantinib resulted in a disease stabilization in a heavily pretreated patient. During a median observation period of 44.5 months, 6 patients died. Median overall survival was not reached.ConclusionPresacral NEN are histopathologically similar to rectal NENs. Presacral NEN should be considered as possible primary in NEN of unknown primary. The majority of tumors is non-functioning and somatostatin receptor positive. PRRT demonstrated promising activity; tyrosine kinase inhibitors warrant further investigations. Further molecular characterization and prospective evaluation of this rare tumor entity are needed.

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Section: Discussionmentioning

confidence: 63%

Multicenter Analysis of Presacral Neuroendocrine Neoplasms—Clinicopathological Characterization and Treatment Outcomes of a Rare Disease

et al. 2021

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“…The presacral lesion is usually a benign entity such as anterior meningocele, teratoma, hamartoma, enteric cyst, or dermoid/epidermoid cyst. Malignant presacral mass degeneration is rare, approximately 1% of all cases with neuroendocrine tumor predominance 4–8 . Cross-sectional imaging features of CS are well known with a presacral mass associated with sacral malformations also known as “scimitar sacrum” or “sickle-shaped sacrum.” 3 In instances of rising concern for malignant degeneration, PET/CT provides the ability to assess the metabolism and degree of aggressive behavior of the presacral lesions, to detect the extent of their potential metastasis, and to monitor their therapeutic response 7,8 …”

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confidence: 99%

18F-FDG PET/CT of Malignant Presacral Masses in Currarino Syndrome

Nguyen¹

2021

Clin Nucl Med

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Currarino syndrome in an elderly man: Multimodality imaging findings

Vitale

Villa

Napoli

et al. 2020

Radiology Case Reports

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Currarino syndrome is a rare congenital disorder characterized by the triad of anorectal anomalies, sacrococcygeal dysgenesis and presacral mass. Because of the anorectal anomalies, the extrinsic compression due to the presacral mass and neurologic deficits, patients usually present with gastrointestinal symptoms, most commonly chronic constipation. Most cases of Currarino syndromes are diagnosed in childhood, at birth or in the pre-birth period and, even if adult presentation has been reported in few sporadic case reports, the diagnosis in the late stages of life remains extremely rare. In this paper, we describe the imaging findings of an elderly man with a past medical history of megacolon surgically treated in his childhood, who was diagnosed with Currarino syndrome at the age of 72.

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Adenocarcinoma and neuroendocrine tumor arising within presacral teratoma associated with Currarino syndrome: A case report

Cited by 4 publications

References 24 publications

Multicenter Analysis of Presacral Neuroendocrine Neoplasms—Clinicopathological Characterization and Treatment Outcomes of a Rare Disease

Multicenter Analysis of Presacral Neuroendocrine Neoplasms—Clinicopathological Characterization and Treatment Outcomes of a Rare Disease

18F-FDG PET/CT of Malignant Presacral Masses in Currarino Syndrome

Currarino syndrome in an elderly man: Multimodality imaging findings

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