first case reported in the trachea
Kurtuluş AKSU1, Zafer AKTAŞ1, Funda AKSU1, Osman ÖRSEL1, Funda DEMİRAĞ2, Tuğrul ŞİPİT1, Selma FIRAT GÜVEN1
1Atatürk Göğüs Hastalıkları ve Göğüs Cerrahisi Eğitim ve Araştırma Hastanesi, Göğüs Hastalıkları Kliniği,
2Atatürk Göğüs Hastalıkları ve Göğüs Cerrahisi Eğitim ve Araştırma Hastanesi, Patoloji Bölümü, Ankara.
ÖZET
Spindle hücreli sarkomatoid karsinom: Trakeada bildirilen ilk olgu
Yetmiş sekiz yaşında erkek hasta üç aydır devam eden öksürük, nefes darlığı, kilo kaybı ve hemoptizi şikayetleri ile başvur- du. Hastanın 30 paket/yıl sigara içme öyküsü ve çevresel asbest maruziyeti mevcuttu. Akciğer tomografisinde karina düze- yinde 2 x 1 cm endolüminal kitle görünümü mevcut olup, mediastinal lenf nodu, parankimal tutulum yoktu. Yapılan fiberop- tik bronkoskopide ana karinaya oturan, sol ana bronş girişini %90’a yakın, sağ ana bronş girişini %75’e yakın daraltan poli- poid tümöral doku izlendi. Endoskopik argon plazma koagülasyon uygulanarak biyopsiler alındı ve lezyonun %90’dan fazla- sı rezeke edilerek hava yolu açıklığı sağlandı. Mikroskobik incelemede tümörün atipik mitotik figürler ve belirgin nükleer po- limorfizm gösteren iğsi hücrelerden oluştuğu ve immünhistokimyasal çalışmada epitelyal membran antijen ile pozitif boyan- ma gösterdiği izlendi. Bu bulgularla olguya spindle hücreli sarkomatoid karsinom tanısı koyuldu. Baş-boyun bölgesi, larenks, gastrointestinal sistem, meme, genitoüriner sistem, cilt ve akciğer olmak üzere pek çok bölgeden gelişen spindle hücreli sar- komatoid karsinom olguları literatürde yer almakla birlikte trakeal kökenli bildirilen tümör olgusu bulunmamaktadır. Trake- anın primer malign tümörleri solunum sisteminde gelişen tümörlerin %0.2’sini oluşturmaktadır ve nadir olmaları sebebiyle in- sidans, epidemiyoloji, etyoloji ve tedavi yöntemleri ile prognoza etkisi olan faktörler hakkında bilgiler kısıtlıdır. En sık rastla- nan histolojik tipler skuamöz hücreli karsinom ve adenoid kistik karsinomdur. Az rastlanan diğer histolojik tipte malign tra- kea tümörlerini bildiren serilerde spindle hücreli sarkomatoid karsinom olgusu olmaması ve bu tümörlerin nadir olmaları se- bebiyle olgu klinik özellikleriyle birlikte sunulmuştur.
Anahtar Kelimeler: Karsinom, epitelyal membran antijeni, spindle hücreli, trakea.
Yazışma Adresi (Address for Correspondence):
Dr. Kurtuluş AKSU, Söğütözü Caddesi Atatepe Sitesi C Blok No: 71 Söğütözü 06520 ANKARA - TURKEY
e-mail: kurtulusaksu@yahoo.com
Primary malignant tracheal tumours are rare and most frequent histological types encounte- red are squamous cell carcinoma and adenoid cystic carcinoma. Spindle cell sarcomatoid car- cinoma according to the 2004 World Health Or- ganization (WHO) classification of lung tumours is grouped among five histological types of sar- comatoid carcinoma. Herein we report a case complaining of cough and dyspnea who rece- ived the diagnosis of spindle cell sarcomatoid carcinoma with the pathological examination of the endoluminal mass discovered in the trachea.
We report the case due to the rarity of these tu- mours and to date spindle cell sarcomatoid car- cinoma of the trachea has not been reported.
CASE REPORT
A 78-years-old male presented with weight loss of 2 kg, dyspnea and cough of three months’ du- ration. The dyspnea had appeared insidiously and had slowly progressed at the time of presen- tation especially at nights leading to sleep dis- turbance. He also expectorated blood-streaked
sputum for a few times. The patient was an ex- smoker with a 30 pack-years smoking history and environmental exposure to asbestos. His medical history was unremarkable and physical examination was all within normal limits. Blood gas analysis showed mild hypoxemia [arterial oxygen tension (PaO2) 60.4 mmHg]. Pulmonary function tests showed obstructive pattern with forced vital capacity (FVC) 72% (% predicted), forced expiratory volume in one second (FEV1), 58% (% predicted) and FEV1/FVC 60%.
Chest computed tomography (CT) scans de- monstrated an endoluminal carinal mass wit- hout any mediastinal or parenchymal lesions (Figure 1). Bronchoscopic examination reve- aled a mass arising from the posterior wall of the trachea 2 cm proximal to the carina. The lesion extended towards the main carina and obstructed the entrances of the left and right main bronchi (Figure 2). Bronchial washing and biopsy of the mass were performed but were non-diagnostic. To take the advantage of SUMMARY
Spindle cell sarcomatoid carcinoma: first case reported in the trachea
Kurtuluş AKSU1, Zafer AKTAŞ1, Funda AKSU1, Osman ÖRSEL1, Funda DEMİRAĞ2, Tuğrul ŞİPİT1, Selma FIRAT GÜVEN1
1Department of Chest Diseases, Atatürk Chest Diseases and Chest Surgery Training and Research Hospital, Ankara, Turkey,
2Department of Pathology, Atatürk Chest Diseases and Chest Surgery Training and Research Hospital, Ankara, Turkey.
A 78-years-old man presented with weight loss, dyspnea, cough and hemoptysis. He was an ex-smoker with a smoking history of 30 pack-years. Imaging studies revealed an endoluminal carinal mass and a tumoral mass arising from the pos- terior wall of the trachea was observed bronchoscopically. Endoscopic argon plasma coagulation was performed to take biopsies and to regain the airway passage. Recanalisation of the right and left main bronchi and the trachea was achieved together with resection of more than 90% of the tumoural mass. Microscopically, the tumor was composed of fascicular pat- tern of spindle cells with atypical mitotic figures and nuclear pleomorphism, positively reactive to epithelial membrane an- tigen supporting a diagnosis of tumour with epithelial differentiation. These findings supported the diagnosis of a spindle cell sarcomatoid carcinoma of trachea. Spindle cell carcinoma cases are reported in many sites, such as head and neck re- gion, larynx, digestive tract, breast, kidney, genital tract, skin and lung, but to our knowledge, not in trachea. Primary ma- lignant tracheal tumours consist mainly of squamous cell carcinoma and adenoid cystic carcinoma and generally have an aggressive course with poor prognosis. We report the first presentation of a spindle cell sarcomatoid carcinoma of trachea together with the clinical course of the patient.
Key Words: Carcinoma, epithelial membrane antigen, spindle-cell, trachea.
Figure 1. Thorax CT sections demonstrating an endoluminal mass in the trachea obstructing the lumens of the right and left main bronchi.
Figure 2. Endobronchial mass arrising from the pos- terior wall of the trachea resulting in 90% and 75%
luminal obstruction at the left and right main bronchi respectively.
Figure 3. Recanalisation of the right and left main bronchi and the trachea following argon plasma coagulation and the residual tumoral infiltration.
Renkli Renkli
Figure 4. Haematoxylin and eosin-stained tissue section showing spindle cells arranged in bundles.
Oval-shaped tumour cells have hyperchromatic nuclei with pleomorphism and atypical mitotic fig- ures (HE x200).
Renkli
Figure 5. An immunostained tissue section showing EMA positivity (Immunostain x400).
Renkli
regaining the airway passage, endoscopic ar- gon plasma coagulation (APC) was performed to take biopsies for the second time. Recanali- sation of the right and left main bronchi and the trachea was achieved but tumoral infiltration was still present on the posterior tracheal wall (Figure 3). In the evaluation of the bronchial trees with fiberoptic bronchoscopy in the same session no endobronchial lesion was present elsewhere. After the procedure dyspnea of the patient almost disappeared and his nocturnal symptoms resolved.
Microscopic examination of the biopsy speci- mens of the mass revealed fascicular pattern of spindle cells with atypical mitotic figures and nuclear pleomorphism (Figure 4,5). The tumour cells were positively reactive to epithelial membrane antigen (EMA) and vimentin where- as immunostain for S-100, CD1a, pankeratin and CD31 were negative. These findings sup- ported the diagnosis of a spindle cell sarcoma- toid carcinoma of trachea.
Physical and radiography examinations of the patient did not reveal any other primary tumours elsewhere in the body. Accordingly surgical re- section of the residual tumour mass was consi- dered to be the most appropriate approach but the patient did not prefer to be operated. He was treated with cisplatin-based chemotherapy regi- men and radiotherapy (a total dose of 60 Gy).
However, the patient died before the chemoradi- otherapy regimen was completed, approxima- tely five months after the diagnosis.
DISCUSSION
Primary malignant tracheal tumours are rare, accounting for only 0.2% of malignant neop- lasms of the respiratory tract (1). Due to their low incidence, knowledge of the epidemiology, etiology, prognostic factors and methods of tre- atment is limited. Primary tracheal cancer is more common in men [except for the histologic type adenoid cystic carcinoma (ACC)] and most patients are former or current smokers (1,2).
Median age at the time of diagnosis varies wi- dely depending on the histologic type, overall peak incidence is the sixth decade of life (2).
Most frequent presenting symptoms are dysp- nea, hemoptysis, dry cough and hoarseness (3).
The most common histologic type of tracheal
tumours is squamous cell carcinoma (SCC) fol- lowed by ACC together account for about 76% of all primary tracheal tumours (3-5). Surgery with or without irradiation is considered to be the tre- atment of choice (1). Single modality radiothe- rapy or combination chemoraditherapy should be administered in cases of unresectable tumo- ur (3). Brachytherapy, photodynamic therapy and cryotherapy are alternative modalities but they are mainly for palliation and lack longterm benefit (3). Extent of local disease is the most important factor determining the therapy (6).
Primary malignant tracheal tumours other than SCC and ACC are extremely rare. The only seri- es of uncommon tracheal tumours reported is that of Gaissert et al. in 2006 (6). In their retros- pective study over 40 years they report 56 cases of malignant uncommon primary tracheal tumo- ur (6). Both in this study and in others concerning tracheal tumours mostly reported uncommon histologic types are carcinoids, lymphoma, small cell carcinoma, adenocarcinoma, large cell car- cinoma, adenosquamous carcinoma, sarcomas and melanoma (1,5,6). We report a case of pri- mary tracheal spindle cell sarcomatoid carcino- ma which is not reported before in the trachea.
Spindle cell sarcomatoid carcinoma, according to the 2004 WHO classification of lung tumours, is one of the five histologic types of sarcomato- id carcinoma (7). Sarcomatoid carcinomas are a group of poorly differentiated non-small cell carcinomas that contain a component of sarco- ma or sarcoma-like (spindle and/or giant cell) differentiation. It contains five groups: ple- omorphic carcinoma, spindle cell carcinoma, gi- ant cell carcinoma, carcinosarcoma and pulmo- nary blastoma (7). Spindle cell carcinoma con- sist of only spindle-shaped tumour cells.
Spindle cells are malignant cells which are car- cinoma in the origin but show mesenchcymal differentiation. These fusiform cells are positi- vely reactive to one or more epithelial markers including cytokeratins, carcinoembryonic anti- gen and EMA (7).
In our case, complete resection of the tumour was not possible since the patient did not want to undergo operation but by APC more than 90% of the endoluminal tumour was resected. Radologi- cally the tumour was an endoluminal mass wit-
hout prominent invasion outside the tracheal wall, thus we had the possibility to evaluate, not the whole, but major part of the tumour. Whole sections were composed of spindle cells with malignant features and positive immunostaining with EMA distinguished epithelial differentation in the tumour. These findings confirmed a diag- nosis of spindle cell sarcomatoid carcinoma. In spindle cell sarcomatoid carcinoma cases the tu- mour cells may co-express vimentin, cytokera- tin, carcinoembryonic antigen and smooth muscle markers (7). In our case positive staining with vimentin was observed, whereas pankeratin was negative moreover, immunostains for S-100, CD31 and CD1a were negative excluding neuro- nal, vascular and dendritic cell tumors.
Although not demonstrated firmly on large seri- es, studies demonstrate that sarcomatoid carci- nomas of the lung are associated with an agg- ressive course and a poor prognosis (8,9). Sur- gical resection of the tumour is the mainstay of the treatment of tracheal tumours, but we admi- nistered chemoradiotherapy to the patient beca- use the patient absolutely refused operation. Un- fortunately, he did not live long and died a five months after the diagnosis.
Spindle cell sarcomatoid carcinoma cases are reported in many sites, such as head and neck region, larynx, digestive tract, pancreas, breast, kidney, genital tract, skin and lung but to our knowledge, not in trachea (10-21). Accordingly, there is not much knowledge about the course and the treatment of the disease. We will have the chance to gain more data as more cases are reported in the future.
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