Lung carcinosarcoma

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Clin Transl Oncol (2010) 12:303-305 DOI 10.1007/s12094-010-0508-6

C A S E R E P O RT S

Lung carcinosarcoma María Jesús Vidal Losada · Vanessa Bernal Monterde · Beatriz Amores Arriaga · Ana Isabel Ferrer Pérez · Sara Serrano Solares · María Tobeña Puyal

Received: 25 March 2009 / Accepted: 26 September 2009

Abstract Lung carcinosarcoma is a neoplasm of high grade composed of epithelial and mesenchymal cells. It is exceptional; and usually affects men who are smokers, between the fifth and eighth decades of life. Medical treatment, chemotherapy and radiotherapy are not active in this kind of tumour, so surgery is the treatment of choice. Prognosis is poor with survival rates at 6 months around 27%. We describe the diagnostic process and the clinical outcome of a patient with lung carcinosarcoma with several paraneoplastic syndromes.

thelial and mesenchymal components. It is more common in men smoking, between 40 and 70 years old. Median survival is around six months and survival rate at 5 years after diagnosis is 6% [1, 2]. We report a case of lung carcinosarcoma with various paraneoplastic syndromes.

Keywords Carcinosarcoma · Lung neoplasm · Paraneoplastic syndromes

We report the case of a 75-year-old man who had worked in the rubber chemical industry and quit smoking 8 years ago. Medical history: ischaemic cardiomyopathy; congestive heart failure and chronic bronchitis with multiple admissions to hospital in the previous year. Surgical history: inguinal herniorrhaphy, two transurethral resections of recurrent bladder cancer, with adjuvant treatment with intravesical BCG. The rheumatology service had started, four months earlier, a study of symmetrical polyarthralgia in the extremities. He also had ankle and wrist inflammation [3]. He was admitted to the emergency department with presyncope. A physical examination showed a good general state except for the presence of a rash on the thorax [4]. Upon lung auscultation, rhonchus and crackles were detected. Peripheral blood analysis was normal and a chest X-ray showed a parahilar right mass with secondary atelectasis. Arterial gasometry showed hypoxaemia (PO2=63.5 mmHg). The patient was admitted to the internal medicine department. A bronchoscopy revealed a complete blockage of the right intermediary bronchus by a polypoid, soft, white tumour. A pathological study showed a high histological tumour, probably of mesenchymal origin, of sarcoma type [1, 2]. In a CT study (Fig. 1) there were no signs of disease in other organs, so the tumour was classified as T2N0M0 (stage IB), amenable to surgery. The surgical treatment was a lower and middle right bilobectomy with mediastinal lymphadenectomy. The preoperative tumour markers were: SCC (2.40 ng/ml); the rest were normal. The definitive pathological study established the diagnosis of lung carcinosarcoma with endobronchial location. The sarcomatous component was pleomorphic malignant fibrohistiocytoma and the epithelial component was squamous cell carcinoma (Fig. 2). Three lymph nodes were isolated from the levels 4R, 7 and 11R with metastases in 7 and 11R by the epithelial component. Stage was then changed to pT2pN2M0 (stage IIIA).

Introduction Lung carcinosarcoma represents less than 0.3% of all malignant tumours of the lung. Histologically, lung carcinosarcoma is presented as a high-grade malignancy with epi-

M.J. Vidal Losada (쾷) · A.I. Ferrer Pérez · S. Serrano Solares · M. Tobeña Puyal Servicio de Oncología Médica Hospital Clínico Universitario Lozano Blesa Avenida San Juan Bosco, 15 ES-50009 Zaragoza, Spain e-mail: [email protected] V. Bernal Monterde Servicio de Digestivo Hospital Clínico Universitario Lozano Blesa Zaragoza, Spain B. Amores Arriaga Servicio de Medicina Interna Hospital Clínico Universitario Lozano Blesa Zaragoza, Spain

Case report

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Clin Transl Oncol (2010) 12:303-305

Fig. 1 TC: parahilar right mass

Fig. 2 Lung carcinosarcoma. Sarcomatous component: pleomorphic malignant fibrohistiocytoma; epithelial component: squamous cell carcinoma

Before surgical treatment, the patient had an episode of migratory polyarthritis, which was treated with non-steroidal anti-inflammatory drugs, but was difficult to control. At this point antibodies were positive. This polyarthritis disappeared after the surgical resection. After the surgery, the patient had a severe paralytic ileus in the context of treatment with morphine, and upper gastrointestinal bleeding [5, 6], probably due to non-steroidal anti-inflammatory drugs. The patient also had a pseudomembranous colitis episode, with an endoscopic search positive for the toxin Clostridium difficile. He spent two months in hospital, and the adjuvant treatment was delayed due to the repeated complications and poor performance status. Three months after surgery, the patient was again admitted to hospital, to the internal medicine department, because of constitutional syndrome and lower extremity paresis. The electroneuromyography study revealed asymmetric mixed motor neuropathy. The diagnosis was paraneoplastic polyneuropathy [7], so we suspected progression of the tumour. He received treatment with dexamethasone and rehabilitation, and achieved clinical improvement. The patient received domiciliary treatment by the palliative care unit due to poor performance status. Five months after surgery we observed a local recurrence and at this point we continued with symptomatic treatment. The patient died 7 months after the diagnosis.

and glandular (50%) in peripheral lung tumours. The sarcomatous component is independent of the location, the different types being rhabdomyosarcoma, chondrosarcoma or osteosarcoma [10]. Carcinosarcoma can be associated with paraneoplastic syndromes, but most of them are not specifically described. These syndromes often manifest as systemic, skin, kidney, endocrine, haematological or neurological affectations. The mixed histology of the carcinosarcoma determines the location of the metastasis. The carcinomatous component tends to metastasise in regional lymph nodes, and the sarcomatous component to distant organs, such as brain, liver, small bowel and the rest of the digestive tract. The primary treatment is surgery, because results with chemotherapy and radiotherapy have not shown any benefit. However, prognosis of the disease is poor. The survival rate at 6 months is 27% and is directly associated to the tumour size [11]. Our patient had few symptoms and diagnosis was an accidental finding on a chest X-ray. Subsequently, he suffered various syndromes and paraneoplastic complications, which negatively affected his quality of life. The first symptom was a seropositive migratory polyarthritis and it disappeared with the surgical resection. The upper gastrointestinal bleeding secondary to treatment with non-steroidal anti-inflammatory drugs and the paralytic ileus due to morphine treatment suggested duodenal metastatic disease in the differential diagnosis [5, 6]. There are several cases reported in the literature with this metastatic disease. We also emphasise the paraneoplastic polyneuropathy diagnosis [7]. As our case shows, different paraneoplastic syndromes can be associated with this disease. The publication of new cases and small series help to improve the knowledge of this uncommon disease.

Discussion Carcinosarcomas represent less than 0.3% of all malignant lung neoplasms. Histological study is defined by a mixed sarcomatous and carcinomatous component, it being necessary to find epithelial and mesenchymal tissue (cartilage, bone and skeletal muscle) [1, 2]. It is a relatively uncommon tumour, which affects men smoking. The median age at diagnosis is 65. Clinically, it is no different to other lung tumours and the symptoms depend on the primary location. This location can be central-endobronchial (most often) or peripheral [8, 9]. The carcinomatous component relates to the location: squamous carcinoma (91%) in central lung tumours

Conflict of interest The authors declare that they have no conflict of interest relating to the publication of this manuscript.

Clin Transl Oncol (2010) 12:303-305

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