Primary sarcomas of the lung: a clinicopathological and immunohistochemical study of 14 cases

Abstract

The clinicopathological features and immunohistochemical findings in 14 primary sarcomas of the lung collected over a 30-year-period are presented. This represents one sarcoma per 550 bronchogenic carcinomas undergoing resection in this centre. The study group comprised six leiomyosarcomas, five malignant peripheral nerve sheath tumours, two haemangiopericytomas and one epithelioid haemangioendothelioma. The majority of cases occurred in men (nine males: five females), with mean age at presentation of 54 years for men and 47 years for women. All leiomyosarcomas were seen in men, whereas malignant peripheral nerve sheath tumours showed no particular sex preponderance. Leiomyosarcomas were larger tumours than malignant peripheral nerve sheath tumours, mean tumour diameter 15 cm (range 10-25 cm) compared to 9.5 cm (7-15 cm), respectively. All leiomyosarcomas were situated intraparenchymally whereas two of the five malignant peripheral nerve sheath tumours were endobronchial in site. Extrathoracic metastates were seen at death in two of the six leiomyosarcomas but not in any of the malignant peripheral nerve sheath tumours. Overall survival was 28 months although for the leiomyosarcoma/malignant peripheral nerve sheath tumour group alone survival was 8 months. Tumour grading appeared to be a more useful prognostic factor than tumour site (endobronchial/parenchymal) or tumour size. Haemangiopericytoma and epithelioid haemangioendothelioma were associated with a more favourable prognosis.

Contrast-Enhanced Dynamic Magnetic Resonance Imaging of Primary Pulmonary Leiomyosarcoma

“…Although leiomyosarcoma is the most frequent type of primary pulmonary sarcoma (Janssen et al 1994;Attanoos et al 1996), immunohistochemical staining is essential for the correct classification of sarcomatous lesions (Moran et al 1997).…”

“…Since the present tumor had no connection to large vessels, it was classified as parenchymal type. This type is further subclassified into proximal bronchial and peripheral subtypes (Shimota et al 1991;Ohta et al 1992;Attanoos et al 1996;Moran et al 1997;Takakura et al 2004). The present case belongs to the latter subtype, which is thought to originate from mesenchymal smooth muscle cells or smooth muscle of small bronchioles (Shimota et al 1991;Ohta et al 1992;Attanoos et al 1996;Moran et al 1997;Takakura et al 2004).…”

“…It is further classified into bronchial and peripheral types by its location and origin (Hartman and Shochat 1983;Shimota et al 1991;Ohta et al 1992;Attanoos et al 1996;Moran et al 1997;Silvestris et al 2003).…”

Evolución posquirúrgica de 7 sarcomas pulmonares primitivos

“…No obstante, la citología es poco fiable y se necesita recurrir a técnicas de inmunohistoquímica, de microscopia electrónica o citoestructurales para hacer posible su diferenciación histológica 10 . La mediastinoscopia está justificada en los casos de sospecha de invasión ganglionar o cuando se cree que existe afectación tumoral mediastínica 6,9 . La cirugía es el mejor método de tratamiento y en la bibliografía se recogen intervenciones quirúrgicas ampliadas en cavidades cardíacas y pared 11,12 , incluso con derivación cardíaca, puesto que la resección es el mejor método de erradicar estos tumores 13 .…”

Primary Endobronchial Leiomyosarcoma

“…Survival after excision of the tumor is better than that of primary lung cancer [11]. The prognosis of tumor is related to the degree of differentiation of tumor and its stage [12]. …”