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Poorly differentiated laryngeal neuroendocrine neoplasm with high serum calcitonin level, a case report, with literature review.

ABSTRACT: Neuroendocrine neoplasm (NEN) of the larynx consists of 0.6% of laryngeal cancer and is the second most common type after squamous cell carcinoma (SCC). Laryngeal NEN rarely secret calcitonin and should be differentiated from medullary thyroid carcinoma. It makes a diagnostic and therapeutic challenge. We describe a case of a laryngeal NEN with calcitonin hypersecretion. A 59-year-old man presented to our clinic with recurrent cough, dysphonia, hoarseness, cervical mass, and significant weight loss. Diagnostic workup showed a supraglottic mass. Biopsy of the lesion revealed large-cell neuroendocrine neoplasm. Further diagnostic workup showed elevated serum calcitonin level. The patient underwent total laryngectomy, thyroidectomy, and modified radical neck dissection. During his follow-up, new subcutaneous nodules appeared that were biopsy-proven metastases. Then adjuvant chemoradiotherapy was performed. Laryngeal NEN with hypersecretion of calcitonin is a rare entity. In patients with elevated serum calcitonin levels and head and neck tumors, it should be considered a differential diagnosis of medullary thyroid carcinoma. As the management and prognosis of these two neoplasms are entirely different.

SUBMITTER: Motiee-Langroudi M

PROVIDER: S-EPMC9780421 | biostudies-literature | 2022 Dec

REPOSITORIES: biostudies-literature

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Poorly differentiated laryngeal neuroendocrine neoplasm with high serum calcitonin level, a case report, with literature review.

Motiee-Langroudi Maziar M Farahzadi Athena A Ansari Reza R Saffar Hana H Darabi Rezvan R Abbasi Mehrshad M

Clinical case reports 20221222 12

Neuroendocrine neoplasm (NEN) of the larynx consists of 0.6% of laryngeal cancer and is the second most common type after squamous cell carcinoma (SCC). Laryngeal NEN rarely secret calcitonin and should be differentiated from medullary thyroid carcinoma. It makes a diagnostic and therapeutic challenge. We describe a case of a laryngeal NEN with calcitonin hypersecretion. A 59-year-old man presented to our clinic with recurrent cough, dysphonia, hoarseness, cervical mass, and significant weight l ...[more]

PMID: 36578797

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Project description:BackgroundLaryngeal neuroendocrine tumors (NETs) represent less than 1% of all malignancies originating from the larynx and available data are limited on case reports. Calcitonin secreting laryngeal NETs are extremely rare and serial dosing of calcitonin in these patients might reveal early relapse or persistence.Case descriptionWe report the case of a 71-year-old woman with persistent pharyngodynia who underwent surgery for an initial diagnosis of small cell undifferentiated neuroendocrine carcinoma (SCUNC) of the larynx (on the epiglottis extended to the left glosso-epiglottic vallecula). The immunohistochemical profile showed the presence of synaptophysin, neuron-specific enolase (NSE), chromogranin A, pan-cytokeratin, including cytokeratin AE1-AE2, and focally calcitonin. The circulating NSE was 13.4 microg/L (normal level <12.5 microg/L) and the basal serum level of calcitonin was 237 pg/mL (normal level <11.5 pg/mL). The patient was started on first-line carboplatin-etoposide chemotherapy because of early relapse to an axillary lymph node. After 4 cycles of treatment, a radiological stability and metabolic response were demonstrated together with a drastic decrease of circulating serum level of calcitonin (from 237 to 57.9 pg/mL). During the follow up, locoregional relapse of disease occurred, associated with an increase of serum calcitonin (89.3 pg/mL). Disease further progressed on and rechallenge with platinum-etoposide chemotherapy was administered, during which clinical progression was confirmed. Due to the lack of response, a revision of the histology was performed and concluded for a definitive diagnosis of moderately differentiated G2 NET, with a Ki-67 index of 22.6%.ConclusionsThis is the eighth case report of laryngeal NET, highlighting the challenge in pathological differential diagnosis and therapeutic strategies. The association with elevated serum calcitonin and the trend of this parameter during clinical progression suggest a role of this marker in the diagnosis and early identification of recurrent laryngeal NETs.

Project description:In the present study, a 36-year-old male presented with left scrotal enlargement without an obvious cause, accompanied by a feeling of heaviness. Imaging examinations revealed a left testicular malignancy, the patient underwent left testicular mass removal,and the postoperative pathology results revealed a highly malignant germ cell tumor, with a tendency toward poorly differentiated embryonal carcinoma or seminoma. After surgery, the condition of the patient deteriorated rapidly, and distant tumor metastasis occurred. Lymph node puncture pathology results revealed poorly differentiated carcinoma consistent with SMARCB1/INI-1 deletion. Despite the use of chemotherapy, radiotherapy, immunotherapy and targeted therapy, the patient died 11 months after surgery. To the best of our knowledge, this is the first case report of a SMARCB1/INI1-deficient Poorly differentiated testicular carcinoma, which is very similar to testicular spermatocytic carcinoma in clinical diagnosis and deserves differentiation for future clinical diagnoses.This report provides important insights into the diagnosis and treatment of SMARCB1/INI1-deficient testicular malignancy. SMARCB1 is a crucial tumor suppressor gene, and its deficiency is closely associated with the development of various malignant tumors. The identification of this case suggests that future research should further explore the molecular mechanisms of SMARCB1-deficient tumors, particularly their role in testicular malignancies. Additionally, the diagnostic process of this case highlights that SMARCB1/INI1-deficient tumors can be clinically very similar to spermatocytic carcinoma of the testis, which can easily lead to misdiagnosis. Therefore, future clinical practice should emphasize the detection of SMARCB1/INI1 expression status, especially in the context of highly aggressive and rapidly progressing testicular malignancies, where immunohistochemical testing for SMARCB1/INI1 should be considered to confirm the diagnosis. In terms of treatment, this case demonstrates the highly aggressive nature and resistance to conventional therapies of SMARCB1/INI1-deficient tumors. Despite the patient receiving multiple treatments, disease progression could not be halted. This underscores the need for the development of novel therapeutic strategies targeting SMARCB1/INI1-deficient tumors, such as combinations of immune checkpoint inhibitors and targeted therapies, or other emerging immunotherapeutic approaches. Moreover, the treatment course of this patient also reflects the importance of individualized treatment plans. Future research should further explore precision medicine strategies based on tumor genetic profiles to improve patient survival rates and quality of life.

Dataset Information

Poorly differentiated laryngeal neuroendocrine neoplasm with high serum calcitonin level, a case report, with literature review.

Publications

Poorly differentiated laryngeal neuroendocrine neoplasm with high serum calcitonin level, a case report, with literature review.

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