Large cell neuroendocrine carcinoma of the lung presenting as pseudoachalasia: a case report

Abstract

IntroductionPseudoachalasia is a rare disease that accounts for only a small percentage of patients with dysphagia. Neuroendocrine tumors are rare malignancies that most commonly originate within the gastrointestinal tract, with the next most common site being the lungs. Esophageal neuroendocrine tumors are the least common site within the gastrointestinal tract. Pseudoachalasia can be secondary to a malignant process within the body. Its typical characteristic in elderly patients is a short duration of symptoms with substantial weight loss.Case presentationA 68-year-old woman presented with worsening dysphagia that had started six months after the resection of a large cell neuroendocrine carcinoma of the lung in 2011. An extensive work-up in 2012, including esophagogastroduodenoscopy, chest computed tomography and positron emission tomography, was unremarkable. Esophageal manometries revealed findings characteristic of achalasia. A repeat esophagogastroduodenoscopy in January of 2014 revealed a nearly circumferential ulcerated, fungating mass in her distal esophagus. Biopsy results confirming a recurrence of her large cell neuroendocrine carcinoma.ConclusionWe report a case of pseudoachalasia due to metastatic large cell neuroendocrine carcinoma of the lung. Our patient had an exceptionally prolonged duration of symptoms preceding the local esophageal recurrence, which was eventually revealed via endoscopy.

Somatostatin Analogs in Clinical Practice: A Review

“…Neuroendocrine tumors (NETs) are a heterogeneous group of tumors that originate in neuroendocrine cells from endocrine glands, endocrine islets, and endocrine cells dispersed throughout the digestive and respiratory tract. The gastrointestinal tract (62-67%) and the lungs (22-27%) are the most common primary tumor sites and the majority are sporadic [88]. GEP-NETs commonly express SSTR [88].…”

“…The gastrointestinal tract (62-67%) and the lungs (22-27%) are the most common primary tumor sites and the majority are sporadic [88]. GEP-NETs commonly express SSTR [88]. Approximately 80% of NETs of the gastrointestinal tract express SSTR2 [89].…”

Incidence of Gastrointestinal Neuroendocrine Tumor: Case Series, Armed Forces Hospital Southern Region, Hospital-Based Tumor Board Registry

“…As neuroendocrine cells are distributed among the whole body, so NETs have been described in multiple organs such as the central nervous system, respiratory tract, the larynx, gastrointestinal (GI) tract, thyroid, skin, breast, and urogenital system. The GIT and lung are the most common primary sites for NET [ 1 , 2 ]. This type of neoplasia has substantial variations in both tumor biology and clinical presentation; the biology of each NET depends on its primary tumor localization, cellular morphology, and mitotic activity, and clinically, NET may manifest by the expression of autonomous hormone secretion of either a peptide hormone or biogenic amine [ 4 ].…”

“…Generally, Neuroendocrine tumors (NETs) are rare tumors comprising ~2% of all malignancies [ 1 ] with the gastrointestinal tract and the lung as the most common sites [ 2 ].…”

Clinicopathological Spectrum of Primary and Metastatic Neuroendocrine Neoplasms

“…By virtue of the body-wide distribution of neuroendocrine cells, NENs have been observed in almost all anatomical locations, such as the GI tract, respiratory tract, central nervous system, thyroid, skin, breast, and urogenital system [ 3 ]. However, the most common sites for primary NENs include the GI tract, pancreatobiliary tract, and lungs [ 4 , 5 ].…”