|Year : 2014 | Volume
| Issue : 2 | Page : 95-97
Primary pulmonary fibrosarcoma: A case report from a Teaching Hospital in Northern Nigeria
Vincent Y Shidali, Mukhtar A Adeiza
Department of Medicine, Ahmadu Bello University Teaching Hospital, Shika-Zaria, Kaduna State, Nigeria
|Date of Web Publication||16-Mar-2015|
Vincent Y Shidali
Department of Medicine, Ahmadu Bello University Teaching, Hospital, PMB 06, Shika-Zaria
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Primary pulmonary fibrosarcomas are rare malignant tumors of mesenchymal origin. We report a case of primary pulmonary fibrosarcoma in a 35-year-old man who presented with chronic cough and progressive breathlessness. Further clinical evaluation revealed a right lung mass extending from the base of the lung to the mid zone. Histology of biopsied sections of the mass confirmed a lung fibrosarcoma thought to be primary in view of the absence of any other source. Outcome was however unfavorable due to death of the patient. Late presentation and the extensive nature of the disease were identified as the cause of mortality. This case is reported because of its rarity.
Keywords: Lung mass, primary pulmonary fibrosarcoma, treatment
|How to cite this article:|
Shidali VY, Adeiza MA. Primary pulmonary fibrosarcoma: A case report from a Teaching Hospital in Northern Nigeria. Ann Nigerian Med 2014;8:95-7
|How to cite this URL:|
Shidali VY, Adeiza MA. Primary pulmonary fibrosarcoma: A case report from a Teaching Hospital in Northern Nigeria. Ann Nigerian Med [serial online] 2014 [cited 2021 May 6];8:95-7. Available from: https://www.anmjournal.com/text.asp?2014/8/2/95/153362
| Introduction|| |
Primary pulmonary fibrosarcomas are malignant tumors of the lungs. They arise from the bronchal wall, lung parenchyma , or pulmonary artery, and thus present as endobronchial or intra-parenchymal mass lesions.
Fibrosarcomas of the lungs are said to be rare and account for <0.05% of all primary lung malignancies,  and 12% of all cases of primary sarcomas of the lungs.  It is also estimated that primary lung sarcomas occur once in every 500 cases of primary lung malignancies. 
With very few patients reported in the literature, very little is known about their clinical behavior and treatment. Diagnosis has to be early and by exclusion of other spindle cell proliferations and sarcomatous neoplasms having similar morphological appearance. ,
| Case Report|| |
The patient is a 35-year-old man who was referred to our unit following a failed attempt at chest tube drainage of a suspected right sided pleural effusion. He had developed cough 6 months prior to presentation and progressive breathlessness in the last 4 months.
The cough was productive of whitish sputum with blood stains, and had started 2 weeks prior to the time of this assessment. He had an associated right sided dull chest pain. Two months into the illness, he noticed progressive breathlessness with reduced exercise tolerance. No paroxysmal nocturnal dyspnea, orthopnea, or body swelling. No history of change in bowel habit, yellowish discoloration of the eyes, weight loss, or fever. He has never smoked cigarette and has never been exposed to organic or inorganic dust. Physical examination revealed a well-preserved young man who was not in any respiratory distress. There was reduced chest movement on the right hemithorax; as well as a dull percussion note and absent breath sounds on the right mid and lower zones. No wheezes or crepitations were heard.
His chest radiograph revealed a right intra-thoracic mass involving the lower two-thirds of the hemothorax, and a restrictive pattern on spirometry. A chest ultrasound showed moderate fluid collection within the right pleural space. Chest computed tomography (CT) scan revealed a solid right lung mass measuring 17 cm × 12.5 cm and extending from the mid zone to the lung base. This was confirmed at surgery by the presence of an extensive tumor consisting of a mixture of solid and liquefied portions with local spread to the chest wall, diaphragm and mediastinum.
Histology of the biopsied sections showed a cellular tumor composed of spindle cells, in a mainly herring-bone pattern. They had a hyperchromatic, tapered, fairly uniformed nuclei, and variable amounts of fibrillary cytoplasm. The stroma was fibrocollagenous and vascularized. Features were in favor of fibrosarcoma. Other investigation results were not remarkable.
A right pneumonectomy was undertaken but the patient died from intra-operative complications.
| Discussion|| |
Fibrosarcomas are potentially highly malignant tumors originating from mesenchymal cells and occurring in various anatomic sites including the lung and chest wall. ,, They may be discovered incidentally during diagnostic work up for unrelated conditions. However, these tumors may also present clinically with chest pain, hemoptysis, cough, fever, chills, and fatigue as in the case under study.  In children where it presents at any age or as primary malignant congenital tumors, similar symptoms may be seen, in addition to serious respiratory distress at birth in the form of status asthmaticus, respiratory distress syndrome, fetal asphyxia; or fetal anemia and relapsing or noncurable pneumonia. ,,,
Initial physical examination leaves an impression of a fluid collection in the pleural space owing to decreased breathe sounds, dull percussion notes, absent wheezes and crepitations, as in the case under consideration. This finding may not be unconnected with the ongoing liquefaction of part of the tumor as found during surgery; however, such findings which are inconsistent with pneumonic lung changes and at the same time not in keeping with pleural effusion on a chest radiograph [Figure 1], should raise some suspicion of a solid mass. This accounted for the initial attempt at chest tube insertion in this patient before referral.
|Figure 1: Chest X-ray (Posterior-anterior view) showing the tumor in the right|
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The extensive nature of the tumor [Figure 1], [Figure 2] and [Figure 3] with invasion into the chest wall and mediastinum as depicted in the CT scan [Figure 2] and [Figure 3] was confirmed at surgery with attachment of the tumor to the diaphragm, and hemorrhagic pericardial effusion. However, this extensive nature of the disease was not consistent with the normal findings on cardiovascular examination, and thus makes a case for the need for routine cardiac imaging, as well as extensive mediastenal nodal evaluation to rule out locoregional metastasis in these groups of patients. 
Histological immunohistochemical evaluation and electron microscopy of the tumor cells were available are critical to the diagnosis of the tumor. The tumors are usually highly cellular, consisting of spindle cells with fusiform nuclei arranged in herring-bone or broad fasicular pattern  as in this patient.
|Figure 2: Computed tomography (sagittal views) showing the tumor extending|
into the chest wall, diaphragm and mediasternum
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|Figure 3: Computed tomography (cross-sectional view) showing tumor|
extension into the chest wall and mediasternum
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In addition, immunohistochemistry is extremely helpful in differentiating fibrosarcomas from other similar tumors in the chest (fibrous mesotheliomas, malignant fibrous tumors of the pleura; other sarcomas such as myxofibrosarcoma, synovial sarcoma or nerve sheath sarcoma). , The mesotheliomas always stain positive for keratin, whereas fibrosarcomas, fibrous tumors of pleura, and most other sarcomas remain negative for keratin and stain positive for vimentine. Other markers such as S-100 protein, desmin or smooth muscle actin allow further differentiation between different types of sarcomas. , For the immunohistochemical diagnosis of carcinomas, the expression of cytokeratin 7 (CK7), CK20, cyclooxygenase 2, thyroid transcription factor-1, chronogranin, synaptophysin, cluster of differentiation 56, prostate-specific antigen, cancer antigen 125, p53, c-erbB-2 proto-oncogene, matrix metalloproteinase 9 and vascular endothelial growth factor serve as useful markers. 
A surgical resection of affected lobes was not successful on the patient due to extensive local spread including to the pericardium, which was not clearly discernable before surgery. Actually, prognosis has been correlated with tumor size, histologic grade, mitotic count, and the location of the tumor (endobronchial vs. intra-parenchymal). ,, In one case series, all patients with a primary fibrosarcoma of the lungs larger than 5 cm in diameter eventually died of the tumor.  Primary pulmonary sarcomas in general have a markedly worse overall survival compared with soft tissue sarcomas of the extremities.  An aggressive approach to treatment is advocated in view of this, but radiotherapy and chemotherapy are preferred for the unresectable cases.
| Conclusion|| |
This case has shown that carefully diagnosed solid thoracic mass in a young subject with no smoking history should be carefully investigated. When outcomes are suggestive of a sarcoma with no other primary site, surgery may not be necessary if extensive local spread has occurred.
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[Figure 1], [Figure 2], [Figure 3]