Citations
Lymphangioleiomyomatosis (LAM) is a slowly progressive neoplastic disease that predominantly affects females. Usually, LAM affects the lung; it can also affect extrapulmonary sites, such as the mediastinum, the retroperitoneum, or the lymph nodes, although these locations are rare. A localized form of LAM can manifest as extrapulmonary lesions; this form is referred to as extrapulmonary lymphangioleiomyoma (E-LAM). Due to the rare occurrence of E-LAM and its variable, atypical location, E-LAM is often difficult to diagnose. Herein, we report the clinicopathological information from four E-LAM cases, and also review previous articles investigating this disease.
Four patients with E-LAM were identified at the Samsung Medical Center (Seoul, Korea) from 1995 to 2012. All E-LAM lesions underwent surgical excision.
All patients were females within the age range of 43 to 47 years. Two patients had para-aortic retroperitoneal masses, while the other two patients had pelvic lesions; two out of the four patients also had accompanying pulmonary LAM. In addition, no patient displayed any evidence of tuberous sclerosis. Histologically, two patients exhibited nuclear atypism with cytologic degeneration.
E-LAM should be considered in the differential diagnosis of patients presenting with pelvic or para-aortic masses. We also conclude that further clinical and pathological evaluation is needed in patients with E-LAM and nuclear atypism.
Citations
Previous studies have suggested an association between usual interstitial pneumonia (UIP) and lung cancer (Ca). However, clinical and histological information is not enough to determine such an association, due to the low incidence and short survival time of patients with both conditions.
We retrospectively reviewed the clinical and histological records of Ca patients with UIP between January 1999 and August 2013 at the Samsung Medical Center, Seoul, Korea. We found 43 patients who had Ca with UIP (UIP-Ca). Previously reported data of eighty-four patients with UIP-only were included as a comparison group.
Smoking is related to poor prognosis in patients with UIP-Ca, and the number of patients with a high smoking index of more than 30 pack-years significantly increased in UIP-Ca patients compared with UIP-only patients. There is no significant prognostic differentiation between UIP-Ca patients and UIP-only patients. Microscopically, UIP-Ca patients showed characteristically heterogeneous histological patterns and degrees of differentiation. There were many foci of squamous metaplasia or dysplasia at the peripheral area of squamous cell carcinomas.
We report 43 cases of UIP-Ca. Our results suggest that smoking is related to cancer occurrence in UIP patients and poor prognosis in UIP-Ca patients.
Citations
It has long been recognized that bronchial schwannomas are extremely rare. As such, diagnosing tumors in this extraordinary location can sometimes be problematic.
We reviewed seven cases of bronchoscopically or surgically resected endobronchial schwannomas and evaluated their clinical and pathologic features.
The present study included five female and two male patients, with ages ranging from 16 to 81 years (mean age, 44.9 years). The clinical presentation varied according to tumor size and location. Patients with more centrally (trachea or main bronchus) located tumors experienced respiratory symptoms (80%) more often than patients with more peripherally (lobar or segmental bronchus) located tumors (0%). Histologically, the tumors were composed of spindle cells that stained with S100 protein. Some of the tumors showed typical Antoni A areas with Verocay body formation. Five of six patients (83.3%) underwent complete tumor removal by rigid bronchoscopy.
Pathologists should consider endobronchial schwannoma in the differential diagnosis of a spindle cell tumor involving the bronchus. Additionally, our results showed that rigid bronchoscopy is an effective tool for tumor removal in endobronchial schwannoma patients.
Citations