Endobronchial myofibroblastic sarcoma presenting with hemoptysis in a patient with a normal chest x-ray.

Abstract: Hemoptysis is an important symptom in clinical practice. The diagnosis of the underlying cause is often difficult, particularly in patients presenting with a normal chest x-ray. We report a case of hemoptysis with a normal chest x-ray due to a rare endobronchial neoplasm: myofibroblastic sarcoma.

Key Words: hemoptysis, myofibroblastic sarcoma, pulmonary disease, x-ray, computed tomography

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Hemoptysis, a relatively common and important symptom, is usually initially evaluated by chest x-rays. We report a case of endobronchial myofibroblastic sarcoma as a rare cause of hemoptysis and detected by computed tomography (CT) with normal chest x-ray. To the best of our knowledge, this is the first reported case of this entity.

Case Report

A 26-year-old man presented with a one-month history of cough, hemoptysis, and dyspnea. He was initially treated with antibiotics but there was no improvement. There was a smoking history of 12 packs per year. Neither cardiac diseases, venous thromboembolism, nor occupational lung disorder were reported. Ear, nose, and throat examination showed mucosal hyperemia of the nose, pharynx, and the oropharynx. Plain chest x-ray was interpreted as normal. Computed tomography (CT) scans of the chest showed a well-defined soft tissue mass 15 by 10 mm in the right main bronchus, and localized precarinal small calcified lymph nodes (Fig. 1). Fiberoptic bronchoscopy demonstrated a bronchial tumor with subtotal occlusion of the right main bronchus. Biopsies were taken, and pathologic examination indicated a malign mesenchymal tumor. Sleeve resection of the right main bronchus was performed. Histopathology documented that the tumor was composed of a mixture of a cell-rich fascicular area and a hypocellular area with myxoid matrix. There were six mitotic figures per 10 high-power field. Hemorrhages and necrosis were also evident. Immunohistochemically, the tumor was positive for vimentin, O-13 (cytoplasmic and membranous), and negative for actin, desmin, keratin, S-100, and protein CD34. Given these cellular features, the tumor was interpreted as myofibroblastic sarcoma. The patient was well three months after the operation.

Discussion

Hemoptysis is a relatively common and serious symptom that can be caused by a number of pathologies, the most common causes being neoplasms, chronic bronchitis, and bronchiectasis. (1) A chest x-ray is usually the first investigation performed. However, 20 to 30% of patients have a normal chest x-ray. (2) Studies assessing the role of CT and bronchoscopy in the diagnosis of hemoptysis showed that CT should precede bronchoscopy in patients with hemoptysis and normal or nonlocalizing chest x-rays to optimize detection of abnormalities and direct cytologic and microbiologic sampling. (3)

[FIGURE 1 OMITTED]

Myofibroblasts are mesenchymal cells found in granulation tissue and benign neoplasms including fibromatoses and myofibroblastomas. Myofibroblastic sarcomas are uncommon tumors. There are few reports of them in the literature. The tumors are usually found in the breast, bones, pleura, thyroid gland, and the soft tissues including the subcutaneous, submucosal, intramuscular, mesenteric, and scrotal. (4,5)

Conclusion

Hemoptysis is a common symptom. Diagnostic workup is important since normal chest x-rays may not be decisive in determining the pathology. As reported here, CT may show a rare endobronchial lesion, myofibroblastic sarcoma, as a cause of the hemoptysis, although the chest x-ray is normal.

Everything that irritates us about others can lead to an understanding
of ourselves.
--Carl Jung

Accepted September 30, 2003.

Copyright [c] 2004 by The Southern Medical Association

0038-4348/04/9703-0314

References

1. Hirshberg B, Biran I, Mendel G, Mordechai RK. Hemoptysis: etiology, evaluation, and outcome in a tertiary referral hospital. Chest 1997;112:440-444.

2. Weaver LJ, Solliday N, Cugell DW. Selection of patients with hemoptysis for fiberoptic bronchoscopy. Chest 1989;76:116-119.

3. Set PA, Flower CD, Smith IE, Cahn AP, Twentyman OP, Sheerson JM. Hemoptysis: comparative study of the role of CT and fiberoptic bronchoscopy. Radiology 1993;189:677-680.

4. Montgomery E, Goldblum JR, Fisher C. Myofibrosarcoma: a clinicopathologic study. Am J Surg Pathol 2001;25:219-228.

5. Taccagani G, Rovere E, Masullo M, Christensen L, Eyden B. Myofibrosarcoma of the breast: review of the literature on myofibroblastic tumors and criteria for defining myofibroblastic differentiation. Am J Surg Pathol 1997;21:489-496.

RELATED ARTICLE: Key Points

* Hemoptysis is an important symptom of pulmonary disease.

* The diagnosis of the etiology of hemoptysis is often difficult, especially when the chest radiograph is normal.

* Bronchoscopy following computed tomography may be a useful strategy.

* Myofibroblastic sarcomas, determined for the first time by computed tomography of the chest, are rare tumors.

Ash Koktener, MD, Gulcin Dilmen, MD

From the Department of Radiology, School of Medicine, Fatih University, Emek, Ankara, Turkey

Reprint requests to Ash Koktener, MD, Assistant Professor, Department of Radiology, School of Medicine, Fatih University, P.K. 5, Emek 06510, Ankara, Turkey. Email: [e-mail]