Carcinosarcoma of the lung associated with neurofibromatosis type 1: a case report

Abstract

Neurofibromatosis or von Recklinghausen's disease is the most common inherited syndrome predisposing to neoplasia. Carcinosarcoma is a rare malignant mixed tumor of the lung. Association of carcinosarcoma of lung with Neurofibromatosis-1 is not common. A 57-year-old man presented with history of fever, cough, hemoptysis, breathlessness, weight loss, chest pain. Multiple cutaneous neurofibromas and café au lait spots were revealed by physical examination. A homogeneous opacity was found in the right middle and right upper zone on posterior-anterior chest radiography. A 8x8x7 cm mass that had irregular borders in right upper posterior and apical segment was seen on contrast enhanced chest computed tomography. On bronchoscopy, the lumen of right upper apical segment was obstructed with vegetating tumoral lesion. The biopsy taken from this region was diagnosed as carcinosarcoma by histopathological and immunohistochemical examination.A rare case with carcinosarcoma of the lung and Neurofibromatosis-1 was reported.

Primary sarcomas of the lung: report of five cases and review of the literature

“…The most frequent epitheliel component is squamous carcinoma and sarcomatous compenent is rhabdomyosarcoma. Our two patients also had the two components just like the literature [8,10]. In the literature, fewer than 20 cases are reported until 1995 [11][12][13][14].…”

Lung Cancer Associated with Neurofibromatosis Type I

“…A review from Japan 20 years ago reported only 11 cases of NF-1 with lung cancer [6], and we interestingly were able to find 7 more cases up to now—without including the presented case—also from the Japanese literature [7–13]. Adenocarcinoma was the most frequent histological diagnosis (72.9%) in the first 11 cases reviewed, while the following 6 cases were diagnosed as nonsmall cell cancer (3 cases) [9, 11, 12], small cell cancer (1 case) [10], poorly differentiated cancer (1 case) [7], adenocarcinoma (1 case) [8], and carcinosarcoma (1 case) [13]. Smoking habit was not always mentioned in the reported cases in the literature, but there were some patients that were never smokers as in our case [8, 13].…”

“…Adenocarcinoma was the most frequent histological diagnosis (72.9%) in the first 11 cases reviewed, while the following 6 cases were diagnosed as nonsmall cell cancer (3 cases) [9, 11, 12], small cell cancer (1 case) [10], poorly differentiated cancer (1 case) [7], adenocarcinoma (1 case) [8], and carcinosarcoma (1 case) [13]. Smoking habit was not always mentioned in the reported cases in the literature, but there were some patients that were never smokers as in our case [8, 13]. In our patient, histology revealed a well-differentiated adenocarcinoma, which is also the most common histological type of lung cancer in nonsmokers, who are by far more common in women [14, 15].…”

A Rare Case of Pulmonary Endobronchial Spindle Cell Carcinoma with Liver Metastasis along with Concurrent Cutaneous Neurofibroma

“…Neurofibromatosis with primary lung spindle cell carcinoma is a very rare presentation. 10 Bhargava et al reported one case of malignant transformation of cutaneous neurofibroma to cutaneous spindle cell carcinoma. 11 conclusion Pulmonary spindle cell tumor is a rare condition with a non-specific clinical presentation like non-small cell carcinoma of the lung associated with cutaneous neurofibroma; hence pathological and immunohistochemical staining is needed to differentiate.…”