Multiple Pulmonary Fibroleiomyomatous Hamartoma

Kód výsledku v IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F27283933%3A_____%2F20%3A00007844" target="_blank" >RIV/27283933:_____/20:00007844 - isvavai.cz</a>

Výsledek na webu

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DOI - Digital Object Identifier

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Jazyk výsledku

angličtina

Název v původním jazyce

Multiple Pulmonary Fibroleiomyomatous Hamartoma

Popis výsledku v původním jazyce

Introduction: Multiple Pulmonary Fibroleimyomatous Hamartoma (MPFH) is a rare disease which occurs mainly in women of middle age and older but also men and children can be affected. Surprisingly, as a rule the diagnosis is not definitely confirmed until histological examination. Due to an indolent nature of hamartomas they are always in practice a coincidental finding. Tumors occur without lung lobe predominance. Their size ranges from small up to several centimeter tumors and they are in the amount of from several to hundreds of nodules in one patient. Case Presentation: A 72-year-old patient underwent chest X-ray during preoperative examination before arthroscopy. Eight spherical shadows of a metastatic nature were observed in the clinical image. Thoracoscopic resection showed a surprising MPFH finding and regular CT controls were decided on. After eight months there was even a spontaneous regression of one of the tumors. Discussion: Surgical performance of MPFH is generally indicated after a coincidental chest X-ray finding followed by verified non-specific depiction of metastatic affection on CT. Magnetic resonance and PET do not provide further crucial information. During PET examination tumors are without activity and not even a possible primary tumor is identifiable. If hormonal dependency is confirmed, anti-estrogen therapy should be started or gynecological resection considered. Conclusion: It is recommended to obtain a representative MPFH sample via thoracoscopy as the results of transparietal biopsy usually are not absolute proof (false negativity). If a patient does not have other problems, the size of tumors and their amount do not progress with the passing of time and at the same time hormonal dependency is not confirmed, regular dispensary care by a pneumologist is sufficient. An alternative treatment is repeated re-resection.

Název v anglickém jazyce

Multiple Pulmonary Fibroleiomyomatous Hamartoma

Popis výsledku anglicky

Introduction: Multiple Pulmonary Fibroleimyomatous Hamartoma (MPFH) is a rare disease which occurs mainly in women of middle age and older but also men and children can be affected. Surprisingly, as a rule the diagnosis is not definitely confirmed until histological examination. Due to an indolent nature of hamartomas they are always in practice a coincidental finding. Tumors occur without lung lobe predominance. Their size ranges from small up to several centimeter tumors and they are in the amount of from several to hundreds of nodules in one patient. Case Presentation: A 72-year-old patient underwent chest X-ray during preoperative examination before arthroscopy. Eight spherical shadows of a metastatic nature were observed in the clinical image. Thoracoscopic resection showed a surprising MPFH finding and regular CT controls were decided on. After eight months there was even a spontaneous regression of one of the tumors. Discussion: Surgical performance of MPFH is generally indicated after a coincidental chest X-ray finding followed by verified non-specific depiction of metastatic affection on CT. Magnetic resonance and PET do not provide further crucial information. During PET examination tumors are without activity and not even a possible primary tumor is identifiable. If hormonal dependency is confirmed, anti-estrogen therapy should be started or gynecological resection considered. Conclusion: It is recommended to obtain a representative MPFH sample via thoracoscopy as the results of transparietal biopsy usually are not absolute proof (false negativity). If a patient does not have other problems, the size of tumors and their amount do not progress with the passing of time and at the same time hormonal dependency is not confirmed, regular dispensary care by a pneumologist is sufficient. An alternative treatment is repeated re-resection.

Druh

O - Ostatní výsledky

CEP obor

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OECD FORD obor

30212 - Surgery

Projekt

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Návaznosti

V - Vyzkumna aktivita podporovana z jinych verejnych zdroju

Rok uplatnění

2020

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů