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Primary pulmonary adenoid cystic carcinoma- clinicopathological features and predictors of poor outcome

INTRODUCTION: Primary pulmonary adenoid cystic carcinoma is a rare disease. Its clinico-pathological features, course of disease, therapeutic strategy, and survival data have not been fully elucidated. We aimed to study the clinicopathological characteristics of primary pulmonary adenoid cystic carc...

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Autores principales: Aiman, Aiffa, Zahir, Zafirah, Makhdoomi, Rumana, Farooq, Roudah Binti, Jeelani, Tazeen
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10298806/
https://www.ncbi.nlm.nih.gov/pubmed/37148019
http://dx.doi.org/10.4103/lungindia.lungindia_204_22
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author Aiman, Aiffa
Zahir, Zafirah
Makhdoomi, Rumana
Farooq, Roudah Binti
Jeelani, Tazeen
author_facet Aiman, Aiffa
Zahir, Zafirah
Makhdoomi, Rumana
Farooq, Roudah Binti
Jeelani, Tazeen
author_sort Aiman, Aiffa
collection PubMed
description INTRODUCTION: Primary pulmonary adenoid cystic carcinoma is a rare disease. Its clinico-pathological features, course of disease, therapeutic strategy, and survival data have not been fully elucidated. We aimed to study the clinicopathological characteristics of primary pulmonary adenoid cystic carcinomas in north India. METHODS: This study was a retrospective, single-centre, cohort study. The database of the hospital was searched for a period of seven years to identify all patients with primary pulmonary adenoid cystic carcinoma. RESULTS: Out of 6050 lung tumors, 10 were primary adenoid cystic carcinomas. The mean age of diagnosis was 42 (±12) years. Six patients had the lesion in the trachea, main bronchus or truncus intermedius and four had parenchymal lesions. Seven patients had resectable tumours. Three patients achieved an R0 resection, two achieved R1 resection and two had R2 resection. On histopathological examination, almost all of the patients had cribriform pattern. Only four patients (57.1%) showed positivity for TTF-1 staining. The five-year survival of patients with resectable tumour and un-resectable tumour was 85.7% and 33.3%, respectively (P = 0.01). The predictors of poor outcome included non-operability of the tumour, presence of metastasis at the time of diagnosis, and macroscopically positive tumour margin during surgery. CONCLUSION: Primary pulmonary adenoid cystic carcinoma is a unique and rare tumour that affects relatively younger individuals, males and females, as well as smokers and non-smokers equally. The features of bronchial obstruction are the most common. Surgery is the primary modality of treatment and completely resectable lesions have the best prognosis.
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spelling pubmed-102988062023-06-28 Primary pulmonary adenoid cystic carcinoma- clinicopathological features and predictors of poor outcome Aiman, Aiffa Zahir, Zafirah Makhdoomi, Rumana Farooq, Roudah Binti Jeelani, Tazeen Lung India Original Article INTRODUCTION: Primary pulmonary adenoid cystic carcinoma is a rare disease. Its clinico-pathological features, course of disease, therapeutic strategy, and survival data have not been fully elucidated. We aimed to study the clinicopathological characteristics of primary pulmonary adenoid cystic carcinomas in north India. METHODS: This study was a retrospective, single-centre, cohort study. The database of the hospital was searched for a period of seven years to identify all patients with primary pulmonary adenoid cystic carcinoma. RESULTS: Out of 6050 lung tumors, 10 were primary adenoid cystic carcinomas. The mean age of diagnosis was 42 (±12) years. Six patients had the lesion in the trachea, main bronchus or truncus intermedius and four had parenchymal lesions. Seven patients had resectable tumours. Three patients achieved an R0 resection, two achieved R1 resection and two had R2 resection. On histopathological examination, almost all of the patients had cribriform pattern. Only four patients (57.1%) showed positivity for TTF-1 staining. The five-year survival of patients with resectable tumour and un-resectable tumour was 85.7% and 33.3%, respectively (P = 0.01). The predictors of poor outcome included non-operability of the tumour, presence of metastasis at the time of diagnosis, and macroscopically positive tumour margin during surgery. CONCLUSION: Primary pulmonary adenoid cystic carcinoma is a unique and rare tumour that affects relatively younger individuals, males and females, as well as smokers and non-smokers equally. The features of bronchial obstruction are the most common. Surgery is the primary modality of treatment and completely resectable lesions have the best prognosis. Wolters Kluwer - Medknow 2023 2023-04-28 /pmc/articles/PMC10298806/ /pubmed/37148019 http://dx.doi.org/10.4103/lungindia.lungindia_204_22 Text en Copyright: © 2023 Indian Chest Society https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Original Article
Aiman, Aiffa
Zahir, Zafirah
Makhdoomi, Rumana
Farooq, Roudah Binti
Jeelani, Tazeen
Primary pulmonary adenoid cystic carcinoma- clinicopathological features and predictors of poor outcome
title Primary pulmonary adenoid cystic carcinoma- clinicopathological features and predictors of poor outcome
title_full Primary pulmonary adenoid cystic carcinoma- clinicopathological features and predictors of poor outcome
title_fullStr Primary pulmonary adenoid cystic carcinoma- clinicopathological features and predictors of poor outcome
title_full_unstemmed Primary pulmonary adenoid cystic carcinoma- clinicopathological features and predictors of poor outcome
title_short Primary pulmonary adenoid cystic carcinoma- clinicopathological features and predictors of poor outcome
title_sort primary pulmonary adenoid cystic carcinoma- clinicopathological features and predictors of poor outcome
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10298806/
https://www.ncbi.nlm.nih.gov/pubmed/37148019
http://dx.doi.org/10.4103/lungindia.lungindia_204_22
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