Mixed Neuroendocrine-Nonneuroendocrine Neoplasms (MiNENs) of the Esophagus

Esophageal neuroendocrine neoplasms are extremely rare, and their prognosis is poor. Mixed neuroendocrine non-neuroendocrine neoplasms (MiNENs) are even more rare and are defined as tumors consisting of neuroendocrine carcinoma and either adenocarcinoma or squamous cell carcinoma. We report a rare case featuring endoscopic submucosal dissection (ESD) for an esophageal MiNEN, arising from the ectopic gastric mucosa in the lower thoracic esophagus. A 92-year-old male patient was referred to this hospital for investigation of an esophageal tumor. An endoscopic examination revealed a 10 mm elevated lesion, with 8 mm flat areas on the anal side, within the ectopic gastric mucosa located in the lower thoracic esophagus. ESD was carried out, and a histopathological examination revealed a tubular adenocarcinoma composed of differentiated neuroendocrine cells. Immunohistochemical staining was positive for synaptophysin and negative for chromogranin A. The labeling index of Ki-67 was more than 80%. Based on these results, we diagnosed the lesion as an esophageal MiNEN, arising in the ectopic gastric mucosa of the esophagus. The patient remains alive, without recurrence of cancer, 24 months after ESD.

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“…Only eleven cases of esophageal MiNEN have been reported in the literature [10–13] (Table 1). Ten were in males, with a wide age range.…”

Section: Discussionmentioning

confidence: 99%

“…Ten cases (91%) were treated with surgery, and ESD was performed in only 1 case. In the literature, 10 cases of eleven esophageal MiNEN had an adenocarcinoma component [10][11][12][13]. On the other hand, Hong et al [14] reported that 10% of the mixed type of esophageal NEC was composed with adenocarcinoma.…”

Section: Case Reports In Gastroenterologymentioning

confidence: 99%

Mixed Neuroendocrine Non-Neuroendocrine Neoplasm Arising in the Ectopic Gastric Mucosa of Esophagus

Gushima¹,

Miyamoto²,

Imamura³

et al. 2022

Case Rep Gastroenterol

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“…These tumors, which account for approximately one-quarter of the NENs observed in this localization, show an apparent male predominance and are observed at advanced ages (6th decade) in the distal third of the esophagus[ 2 , 19 , 62 ]. Although the tumor consists of NEC and squamous cell carcinoma (SCC) in most cases, there are rare adenocarcinoma cases, particularly adenocarcinoma, in the background of Barrett's esophagus[ 40 , 63 , 64 ]. Molecular data indicate their monoclonal origin, as demonstrated by LOH, RB1, TP53, and alterations in TP63, SOX2, DVL3, PTEN, PIK3A, and KRAS[ 40 ].…”

Section: Organ-specific Clinicopathological Findingsmentioning

confidence: 99%

“…Molecular data indicate their monoclonal origin, as demonstrated by LOH, RB1, TP53, and alterations in TP63, SOX2, DVL3, PTEN, PIK3A, and KRAS[ 40 ]. However, the monoclonal origin of esophageal MiNEN with a nonendocrine component composed of adenocarcinoma on the background of Barrett’s metaplasia remains to be elucidated, and some authors postulate that this group reflects a true collision tumor rather than MiNEN[ 64 ].…”

Section: Organ-specific Clinicopathological Findingsmentioning

confidence: 99%

Mixed neuroendocrine–nonneuroendocrine neoplasms of the gastrointestinal system: An update

Elpek¹

2022

WJG

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Mixed neuroendocrine-nonneuroendocrine neoplasms (MiNENs) of the digestive tract are a rare heterogeneous group of tumors that present many challenges in terms of diagnosis and treatment. Over the years, the diagnostic criteria, classification, and clinical behavior of these tumors have been the subjects of ongoing debate, and the various changes in their nomenclature have strengthened the challenges associated with MiNENs. This review is performed to provide an understanding of the key factors involved in the evolution of the designation of these tumors as MiNEN, highlight the current diagnostic criteria, summarize the latest data on pathogenesis and provide information on available treatments. Moreover, this work seeks to increase the awareness about these rare neoplasms by presenting the clinicopathological features and prognostic factors that play important roles in their behavior and discussing their different regions of origin in the gastrointestinal system (GIS). Currently, the MiNEN category also includes tumors in the GIS with a nonneuroendocrine component and epithelial tumors other than adenocarcinoma, depending on the organ of origin. Diagnosis is based on the presence of both morphological components in more than 30% of the tumor. However, this value needs to be reconfirmed with further studies and may be a limiting factor in the diagnosis of MiNEN by biopsy. Furthermore, available clinicopathological data suggest that the inclusion of amphicrine tumors in the definition of MiNEN is not supportive and warrants further investigation. The diagnosis of these tumors is not solely based on immunohistochemical findings. They are not hybrid tumors and both components can act independently; thus, careful grading of each component separately is required. In addition to parameters such as the metastatic state of the tumor at the time of diagnosis and the feasibility of surgical resection, the aggressive potential of both components has paramount importance in the choice of treatment. Regardless of the organ of origin within the GIS, almost MiNENs are tumors with poor prognosis and are frequently encountered in the elderly and men. They are most frequently reported in the colorectum, where data from molecular studies indicate a monoclonal origin; however, further studies are required to provide additional support for this origin.

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“…Due to the presence of different proportions of each component these cancers display a wide heterogeneity. Consequently, the prognosis and overall survival of these tumors are variable and depends on the tumor location within the GI tract[ 27 - 29 ].…”

Section: Introductionmentioning

confidence: 99%

Mixed neuroendocrine and adenocarcinoma of gastrointestinal tract: A complex diagnosis and therapeutic challenge

Shenoy

2024

World J Gastrointest Oncol

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In this editorial we comment on the manuscript describing a case of adenocarcinoma mixed with a neuroendocrine carcinoma of the gastroesophageal junction. Mixed neuroendocrine and non-neuroendocrine neoplasms of the gastrointestinal system are rare heterogeneous group of tumors characterized by a high malignant potential, rapid growth, and poor prognosis. Due to the rarity of these cancers, the standard therapy is poorly defined. The diagnosis of these tumors is based on combination of morphological features, immunohistochemical and neuroendocrine and epithelial cell markers. Both endocrine and epithelial cell components can act independently of each other and thus, careful grading of each component separately is required. These cancers are aggressive in nature and the potential of each component has paramount importance in the choice of treatment and response. Regardless of the organ of origin, these tumors portend poor prognosis with increased proportion of neuroendocrine component. Multidisciplinary services and strategies are required for the management of these mixed malignancies to provide the best oncological outcomes. The etiopathogenesis of these mixed tumors remains obscure but poses interesting question. We briefly discuss a few salient points in this editorial.

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Mixed Neuroendocrine-Nonneuroendocrine Neoplasms (MiNENs) of the Esophagus

Cited by 6 publications

References 2 publications

Mixed Neuroendocrine Non-Neuroendocrine Neoplasm Arising in the Ectopic Gastric Mucosa of Esophagus

Mixed Neuroendocrine Non-Neuroendocrine Neoplasm Arising in the Ectopic Gastric Mucosa of Esophagus

Mixed neuroendocrine–nonneuroendocrine neoplasms of the gastrointestinal system: An update

Mixed neuroendocrine and adenocarcinoma of gastrointestinal tract: A complex diagnosis and therapeutic challenge

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