Coexistence of pulmonary sclerosing hemangioma and primary adenocarcinoma in the same nodule of lung

Abstract

Pulmonary sclerosing hemangiomas (PSH) of the lung are uncommon tumors and may present cytological atypia with unusual manifestations. The development of PSH combined with other different tumors in lung is extremely rare. We report a case of coexistence of PSH and primary adenocarcinoma in a young female occurring in the same pulmonary nodular mass of right lower lobe. The solitary mass of lung was well-circumscribed on chest computed tomography (CT) and gross examination. Histologically, the mass contained two separated portions and displayed typically histological features of PSH and acinar adenocarcinoma, respectively. In PSH portion, the tumor was composed of sheets of round cells with scattered surface cuboidal cells forming small tubules. Both round and surface cells were diffusely positive for epithelial membrane antigen (EMA) and thyroid transcription factor-1 (TTF-1), but lack immunoreactivity for pancytokeratin in round cells. In adenocarcinoma portion, the tumor cells formed irregular-shaped glands with cytologically malignant cells infiltrating in fibroblastic stroma, and no TTF-1-positive round cells could be observed in this portion. Under the microscopy, there was no gradual transition of these two portions observed in mass. A diagnosis of PSH combined with primary adenocarcinoma of lung was made. There was no evidence of tumor recurrence during the period of postoperative 6-month follow-up. To our knowledge, this is the first case of coexistence of PSH and adenocarcinoma in the same nodule of lung. In addition, the biological behavior and histological differential diagnosis of this tumor were also discussed.

In pulmonary sclerosing hemangioma expression of β-catenin, Axin, and C-myc differs between the two cell types

“…Dacic et al [20] proposed that PSH has the same origin and a similar molecular pathogenesis as bronchioloalveolar carcinoma, with PSH having a biological behavior similar to BAC. Liu et al [28] recently reported a case of coexistence of PSH and primary adenocarcinoma in the same nodule of lung. This result also suggested that the two tumor types maybe closely related.…”

Multiple peripheral typical carcinoid tumors of the lung: associated with sclerosing hemangiomas

“…These include PSH presenting in combination with: atypical alveolar hyperplasia (AAH), alveolar adenoma, primary pulmonary adenocarcinoma, metastatic hereditary non-polyposis colorectal cancer, and primary adenocarcinoma within the same lobe of the lung [3-6,31]. The authors hypothesized that the AAH or adenocarcinoma-like lesion may be a precursor lesion leading to PSH.…”

“…In this current study, the round cells of the PSH showed immuno-positivity for TTF-1 and were negative for Syp. Literature suggests that the positivity of TTF-1 and Syp indicates cells originating from the primitive respiratory cells [6]. Syp is usually positive in neuroendocrine tumors such as in carcinoid tumors.…”

“…However, the histologic characteristic of PSH is well known to show papillary, sclerotic, solid, and hemorrhagic patterns with two cell types [2]. Multiple studies report cases about PSH with unconventional histological morphologies: PSH presenting in multiple nodules of atypical adenomatous hyperplasia, PSH containing adenocarcinoma-like portion, coexistence of PSH with primary adenocarcinoma, and PSH with metastatic hereditary non-polyposis colorectal cancer [3-6]. However, there has never been a publication about multiple typical carcinoids found simultaneously with sclerosing hemangiomas within the same lobe of the lung.…”

Pulmonary sclerosing hemangioma presenting with dense spindle stroma cells: a potential diagnostic pitfall

“…However, immunohistochemical and molecular findings strongly suggest that PSH originates from primitive respiratory epithelium [4,5,8]. As an epithelial tumor, PSH is histologically distinctive.…”