Dopamine-Secreting Paraganglioma in the Retroperitoneum

Matsuda, Yusuke; Kimura, Noriko; Yoshimoto, Takanobu; Sekiguchi, Yoshihiro; Tomoishi, Junzo; Kasahara, Ichiro; Hara, Yuki; Ogawa, Yoshihiro

doi:10.1007/s12022-016-9457-0

Cited by 9 publications

(10 citation statements)

References 13 publications

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“…Tyrosine hydroxylase, a rate‐limiting enzyme in catecholamine synthesis may show immunoreactivity in functional paragangliomas. However, the expression may be weak to negative in parasympathetic or nonfunctional paragangliomas . Other neuroendocrine markers such as CD56, CD57, PGP9.5, are not as reliable as chromogranin and synaptophysin .…”

Section: Discussionmentioning

confidence: 99%

Peripancreatic paraganglioma mimics pancreatic/gastrointestinal neuroendocrine tumor on fine needle aspiration: Report of two cases and review of the literature

Zeng

Simsir

Oweity

et al. 2017

Diagnostic Cytopathology

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Cytologic diagnosis of extra-adrenal paraganglioma presenting as a peripancreatic mass is challenging with a high error rate due to its rarity. We report two cases of peripancreatic masses identified by radiology. Endoscopic ultrasound-guided fine needle aspiration (FNA) of the masses showed a moderately cellular tumor composed of small to medium sized neoplastic cells with round to oval nuclei, arranged singly and in loose clusters. Focal rosette-like structures were present. The cells were positive for neuroendocrine markers (synaptophysin and chromogranin). A diagnosis of a neoplasm with neuroendocrine differentiation and neuroendocrine tumor was made respectively on FNA for each case. The subsequent surgical resection of the tumors revealed peripancreatic paraganglioma. Although paraganglioma has been reported in the literature, the detailed comparison of perpancreatic paraganglioma versus pancreatic/gastrointestinal neuroendocrine tumor is still lacking. Therefore using these two cases with literature review, we wish to illustrate the differential diagnosis between these two entities based on cytomorphology and immunohistochemical study.

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Section: Discussionmentioning

confidence: 99%

Peripancreatic paraganglioma mimics pancreatic/gastrointestinal neuroendocrine tumor on fine needle aspiration: Report of two cases and review of the literature

Zeng

Simsir

Oweity

et al. 2017

Diagnostic Cytopathology

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“…Tyrosine hydroxylase (TH) and DBH are essential enzymes for catecholamine synthesis and both antibodies are useful for identification of catecholamine-producing tumors. However, some non-functioning PPGLs including only dopamine-producing or SDHB -related PPGLs lack TH immunoreactivity [ 39 , 40 ], but constantly positive for DBH, making DBH a better marker than TH for identification of PPGLs [ 20 , 36 ] ( Figure 1 ). A flowchart for pathologically differential diagnosis of PPGLs is presented ( Figure 2 ).…”

Section: Flowchart For Differential Diagnosismentioning

confidence: 99%

Risk Stratification on Pheochromocytoma and Paraganglioma from Laboratory and Clinical Medicine

Kimura

Takekoshi

Naruse

2018

JCM

Self Cite

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Pheochromocytoma (PCC) and sympathetic paraganglioma (PGL) are rare neuroendocrine tumors characterized by catecholamine production in the adrenal medulla and extra-adrenal paraganglia. PCC and PGL (PPGL) with metastasis was termed malignant PPGL. However, the distinction between “benign” and “malignant” PPGLs has been debated. Currently, all PPGLs are believed to have some metastatic potential and are assigned malignant tumors (ICD-O/3) by the WHO Classification of Endocrine Organs (2017, 4th edition). Therefore, the previous categories benign and malignant PPGL have been eliminated in favor of risk stratification approach. The Grading of Adrenal Pheochromocytoma and Paraganglioma (GAPP) is a tool for risk stratification for predicting metastasis and the prognosis of patients. At least 30% of PPGLs are hereditary, with 20 genes identified and genotype-phenotype correlations clarified. Of these genes, VHL, RET and NF1 have been well investigated and are the primary cause of bilateral PCC. In addition, mutation of succinate dehydrogenase gene subunits SDHB and SDHD are strongly correlated with extra-adrenal location, younger age, multiple tumors, metastasis and poor prognosis. Disease stratification by catecholamine phenotype and molecular profiling correlates with histological grading by GAPP. PPGLs should be understood comprehensively based on clinical, biochemical, molecular and pathological data for patient care. A flow chart for pathological diagnosis is included.

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“…16.5). Some experts also combine dopamine hydroxylase (another enzyme involved in the catecholamine synthesis, converting L-DOPA to dopamine) along with tyrosine hydroxylase in the workup of paragangliomas [106].…”

Section: Tyrosine Hydroxylase (Th)mentioning

confidence: 99%

Metastatic Neuroendocrine Neoplasms of Unknown Primary Site

Uccella

Sylvia

Mete

2020

The Spectrum of Neuroendocrine Neoplasia

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Virtually all neuroendocrine neoplasms (NENs) have metastatic potential, and up to 20% of the cases present as metastasis from an occult primary [1][2][3]. The identification of the primary site is an important step toward the correct management of the patient, particularly when dealing with a well-differentiated neuroendocrine tumor (NET), as therapeutic approach may vary depending on the site and cell type. In contrast, poorly differentiated neuroendocrine carcinomas (NECs), independent of the primary site, are currently treated with platinum-based regimens, and the role of the pathologist may be limited to the distinction between a visceral NEC and a Merkel cell carcinoma of the skin, because the latter requires wide local excision, sentinel node biopsy, and, possibly, radiotherapy. In contrast, thorough morphological and immunohistochemical analyses are expected to give important clues to the recognition of the site of origin of a metastatic NET. Among all NETs, the tendency to metastasize is highest for those of pancreatic origin, followed by small intestinal, colonic, pulmonary, and gastric neoplasms [1]. Irrespective of the primary site, the liver represents the most frequent location of

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Dopamine-Secreting Paraganglioma in the Retroperitoneum

Cited by 9 publications

References 13 publications

Peripancreatic paraganglioma mimics pancreatic/gastrointestinal neuroendocrine tumor on fine needle aspiration: Report of two cases and review of the literature

Peripancreatic paraganglioma mimics pancreatic/gastrointestinal neuroendocrine tumor on fine needle aspiration: Report of two cases and review of the literature

Risk Stratification on Pheochromocytoma and Paraganglioma from Laboratory and Clinical Medicine

Metastatic Neuroendocrine Neoplasms of Unknown Primary Site

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