Sclerosing Pneumocytoma with Lymph Node Metastasis

Abstract

A 33-year-old woman with a family history of multiple cancers had been undergoing surveillance scans. Magnetic resonance imaging revealed cysts in the kidney and pancreas, multiple hemangiomas in the liver, and a lesion in the left lower lobe (LLL) of the lung. Repeat positron emission tomogram/computed tomography demonstrated a 1.8 Â 1.6-cm nodule ( Fig. 1) within the LLL.The patient underwent endobronchial ultrasoundguided fine needle aspiration biopsy of the LLL nodule, which showed hypercellular smears containing uniform, cuboidal, round, and oval medium-sized cells. They were diffusely positive for thyroid transcription factor-1, and negative for neuroendocrine cell markers. The diagnosis was well-differentiated lung adenocarcinoma. The patient underwent video-assisted thoracoscopic LLL lobectomy, which showed a well-circumscribed 2.5-cm yellow-tan nodule composed of mostly monomorphic, cuboidal surface cells and round cells with pink to clear cytoplasm and well-defined cell borders in papillary, solid, and sclerotic growth patterns. Occasional hemorrhagic foci were also present. A focal area of mild cytologic atypia was noted. A focus of metastatic tumor deposit containing solid sheets of cells was identified in one of the peribronchial lymph nodes. The results of staining for thyroid transcription factor-1 and epithelial membrane antigen were positive in both surface and round cells, but cytokeratin 7 and cytokeratin AE1/AE3 stained only surface cells (Fig. 2). The cytomorphologic examination findings and immunoprofile were consistent with sclerosing pneumocytoma (SP) with a lymph node metastasis. SP (previously called sclerosing hemangioma) is a rare tumor of the lung that is thought to arise from primitive respiratory epithelium. This tumor usually occurs in middle-aged adults with a male-to-female ratio of 1:5. 1 Surgical resection is curative without a need for additional treatment. Although SP is considered a benign tumor, we found a lymph node (LN) Figure 1. Color-fused positron emission tomography/computed tomography scan axial view demonstrating the left lower lobe mass with elevated 18 F-fludeoxyglucose uptake with a maximum standardized uptake value of 6.1.

Clinical and histopathological features of pulmonary sclerosing pneumocytoma with dense spindle stromal cells and lymph node metastasis

“…We reviewed the literature, and identified 25 PSH cases with lymph node or distant metastasis, including three of the cases reported herein (Table 2). 2,5,[7][8][9][10][13][14][15][16][17][18][19][20][21][22][23][24][25] Although the data for one patient were incomplete, the clinicopathological data of the other 24 patients are shown in Table 2. Twenty-two PSP cases with positive lymph nodes have been reported so far.…”

Clinicopathological and Molecular Characteristics of Atypical Pulmonary Sclerosing Pneumocytoma With Multiple Metastases

“…The malignant progression and metastasis of PSP were extremely rare. The reported PSP patients with LN [9,13,14,23] or organ metastases [11,24] were mostly females, and only one male patient suffered from mediastinal LN metastasis [25]. Herein, we reported an extremely atypical case: a young male patient suffering from an aggressive PSP with multiple LN and organ metastases during 7 months after the resection of the primary lung lesion.…”

“…Occasionally, PSP may manifest as multiple lesions, recurrence, regional lymph node (LN), or single organ metastasis, but does not seem to affect the prognosis. Therefore, PSP is considered as a benign or a low-grade malignant potential primary lung tumor [9][10][11][12][13][14].…”

Sclerosing Pneumocytoma: A Ten-Year Experience at a Western Balkan University Hospital

“…In the largest series of published cases, which included 100 patients, only one patient had a lymph node metastasis [19]. According to other studies, lymph node metastasis is more likely to occur in younger patients, with no influence on a patient’s prognosis [25,26]. The mechanism of the metastasizing of SP is still unclear.…”