Squamous Differentiation in High Grade Large Cell Neuroendocrine Carcinoma of Ampulla of Vater

Purpose: A high rate of response to treatment with epidermal growth factor receptor tyrosine kinase inhibitor (EGFR-TKI) has been observed in certain patients (women, of East Asian ethnicity, with non-smoking history and adenocarcinoma histology) with mutations in exons 18 to 21 of the tyrosine kinase domain of EGFR. Some cases of high-grade neuroendocrine carcinoma of the lung harboring mutations have been sporadically reported. Methods: We describe the case of a 78-year-old woman with large-cell neuroendocrine carcinoma of the lung, with mutation in exon 21 L858R and co-expression of adenocarcinoma markers. Results: A mass (3.0 cm in diameter) was identified in the inferior lobe of the left lung, accompanied by metastases into ipsilateral mediastinal lymph nodes and elevations of serum pro-gastrin-releasing peptide and carcinoembryonic antigen. Initial transbronchial brushing cytology suggested high-grade neuroendocrine carcinoma favoring small-cell carcinoma in poorly smeared and degenerated preparations, and revealed exon 21 L858R mutation. Re-enlargement of the cancer and bone metastases was observed after chemotherapy, and further testing suggested large-cell neuroendocrine carcinoma with immunoreactivity to markers of primary lung adenocarcinoma and L858R mutation. High-grade neuroendocrine carcinoma with mutations in the tyrosine kinase domain of EGFR may be associated with adenocarcinoma, as reviewed from the literature and may also apply to our case. Conclusions: EGFR-TKI could provide better quality of life and survival in patients with advanced or relapsed high-grade neuroendocrine carcinoma with EGFR gene mutations. Further studies in this respect are warranted.

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A rare presentation of an ACTH‐producing high‐grade large cell neuroendocrine carcinoma with Cushing’s syndrome

Clinical Case Reports ◽

10.1002/ccr3.5168 ◽

2021 ◽

Vol 9 (12) ◽

Author(s):

Francis Essien ◽

Christine Persaud ◽

David Dado ◽

Rina Eden ◽

Joshua Tate ◽

...

Keyword(s):

Neuroendocrine Carcinoma ◽

Cushing’S Syndrome ◽

Large Cell ◽

Cushing's Syndrome ◽

Large Cell Neuroendocrine Carcinoma ◽

High Grade ◽

Rare Presentation

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Large Cell Neuroendocrine Carcinoma of the Ampulla of Vater

Archives of Pathology & Laboratory Medicine ◽

10.5858/2003-127-221-lcncot ◽

2003 ◽

Vol 127 (2) ◽

pp. 221-223 ◽

Cited By ~ 2

Author(s):

Alberto Cavazza ◽

Marco Gallo ◽

Roberto Valcavi ◽

Loredana De Marco ◽

Giorgio Gardini

Keyword(s):

Mitotic Index ◽

Neuroendocrine Carcinoma ◽

Poor Prognosis ◽

Neuron Specific Enolase ◽

Large Cell ◽

Ampulla Of Vater ◽

Large Cell Neuroendocrine Carcinoma ◽

Neoplastic Cells

Abstract We report a large cell neuroendocrine carcinoma arising in the ampulla of Vater. The patient, a 74-year-old woman, presented with a 3-cm ulcerated mass located in the ampullary region. She died of disease 8 months after surgery. Microscopically, the tumor was extensively necrotic. It was composed of islands and trabeculae irregularly infiltrating the muscular wall of the duodenum. Neoplastic cells were large and had a high mitotic index. Immunohistochemically, they expressed cytokeratin, chromogranin, synaptophysin, and neuron-specific enolase. Large cell neuroendocrine carcinoma is very rare in the ampulla of Vater, and it shares with its more common pulmonary counterpart the same morphology and probably the same poor prognosis.

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