Mucinous cystadenocarcinoma arising from mucinous cystadenoma of the lung: case report and review of the literature

Abstract

Mucinous cystadenoma is a benign tumor commonly found in the pancreas, the ovaries or the appendix. Only very few cases of these tumors originating from the lungs have been reported worldwide, with even less cases describing malignant transformation. We present the case of a 58-year-old woman with a history of recurrent pulmonary infections who underwent left upper lobectomy for lung abscess and was initially diagnosed with pulmonary mucinous cystadenoma (PMCA). Upon thorough immunohistochemical workup, especially due to carcinoembryonic antigen (CEA) positivity, intramucinous singlet cells were eventually diagnostic for invasive carcinoma, in this case a mucinous cystadenocarcinoma arising from a PCMA. PMCA is a rare benign tumor whose potential for malignant transformation has not yet been fully understood. Due to the low number of cases further studies are needed to evaluate if there is a benefit of complete oncologic resection, provided the general condition of the patient allows it. A review of the currently available literature serves to better understand the clinical, radiological and histological features of this rare tumor.

Pulmonary mucinous cystadenoma complicated with infection

“…The median size was 5 cm (range, 0.8–15 cm). [ 3 ] Although our patient was male, he was nearly 60 years old, with a mass size of approximately 4.6 × 5.3 cm, also located in the right lung, which was generally consistent with the literature reports.…”

“…These IHC features suggest that the tumor is derived from the non-alveolar epithelium. [ 3 , 4 ] A positive CEA indicates that the tumor has a local malignancy or is prone to recurrence after surgery. IHC examination of our patient specimen showed positive CK7 and negative TTF-1 expression, which was consistent with the literature reports.…”

“…Radiographically PMCA appears as a well-defined, homogeneous lesion. [ 3 , 4 ] PMCA is usually well-circumscribed and homogeneous on CT, and the cyst wall is usually thin. Inflammation and adjacent atelectasis only seem to be present after a certain enlargement of the lesion due to compression and distortion of the surrounding tissue.…”

“…[ 7 ] The prognosis for these patients is usually good, with recurrence being rare and metastases from malignant lesions being exceptional. [ 3 , 6 , 7 ] Matsuo et al reported a case of PMCA with tumor recurrence 20 years after surgery, which was the only case of recurrence reported in the literature. [ 7 ] Matsuo et al reported a case of pulmonary mucinous cystadenocarcinoma arising from PMCA, [ 7 ] and Davison et al reported a case of adenocarcinoma arising in a mucinous cystadenoma of the lung.…”

Pulmonary Mucinous Cystadenoma – A Rare Pathology

“…There have been forms of multifocal pulmonary mucinous cystic neoplasia described with ovarian type stroma that appears positive for AE1/AE3, EMA and CK7 and negative for CK20 and CK19 (1). However, pulmonary mucinous cyst adenocarcinoma appears positive for CK7, related homeobox gene 2 (CDX2), CA, TTF-1 and CK20 (2,22,27). In the case of primary mucinous cyst neoplasia, immunohistochemistry is positive for AE1/AE3 and CK7 and negative for CK20 (7).…”