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Synovial Sarcoma and Endobronchial Invasion

Blasco, Miguel MD; Quadrelli, Silvia Adriana MD; Bosio, Martín MD; Smith, Reynaldo MD; Salvado, Alejandro MD; Chertcoff, Julio MD

doi: 10.1097/LBR.0b013e3181757157
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Endobronchial metastases from nonpulmonary tumors are very uncommon and sarcomas are especially unusual. Few primary mammary sarcomas have been reported and metastatic disease at the initial presentation is exceptional. We report an unusual case of complete airway obstruction resulting from distant metastasis of a primary breast sarcoma. A healthy 33-year-old woman, life-long nonsmoker, developed cough and progressive shortness of breath. A chest x-ray revealed a right upper lobe atelectasis with an approximately 6 cm in diameter round lesion as confirmed by a computerized tomography scan. A complete right lung atelectasis developed in 24 hours. Flexible bronchoscopy revealed a polypoid lesion completely occluding the right main bronchus. The tumor was endoscopically excised using endobronchial electrosurgery, initially. Pathologic diagnosis demonstrated undifferentiated sarcoma. A right upper lobectomy was performed and the surgical specimen confirmed diagnosis of synovial sarcoma. Physical examination disclosed a hard lump in the right breast and in the left mammary gland; the latter was proven to be synovial sarcoma. A whole body positron emission tomography scan was also performed which did not show any other sites of metastasis. Metastatic soft-tissue sarcoma should be included in the differential diagnosis of endobronchial metastases.

Respiratory Medicine Unit, British Hospital of Buenos Aires, Argentina

Miguel Blasco: performed diagnostic and therapeutic bronchoscopy.

Silvia Quadrelli: designed and wrote the paper.

Martín Bosio: cooperated in writing and performed the literature review.

Reynaldo Smith and Julio Chertcoff: attending physicians.

There is no conflict of interest.

Reprints: Silvia Adriana Quadrelli, MD, Respiratory Medicine Unit, British Hospital of Buenos, Perdriel 74, Buenos Aires 1147, Argentina (e-mail: silvia.quadrelli@gmail.com).

Received for publication February 27, 2008; accepted March 12, 2008

Endobronchial metastases (EBM) from nonpulmonary tumors are uncommon.1–3 In a retrospective review of 1359 consecutive autopsies, Braman and Whitcomb1 described metastatic involvement of a major airway in only 2% of patients who died with solid tumors. The most common extrathoracic tumors associated with metastatic involvement of a central airway were renal and colorectal carcinomas. Sarcomas were extremely unusual as a source of endobronchial metastasis. In 24 patients who had endobronchial metastatic carcinomas only one had sarcoma and all but one had a past history of malignancy.3

In contrast, few primary mammary sarcomas have been reported; most of them osteosarcomas.4–6 In the only series with several cases of primary breast sarcoma, only one in 50 had metastatic disease at the initial presentation.7

We report an unusual case of complete airway obstruction resulting from distant metastasis of a primary breast sarcoma. This patient had no prior diagnosis of cancer before the endobronchial mass was endoscopically diagnosed and resected using endobronchial electrosurgery.

Only postresection work-up revealed the primary breast tumor.

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CASE REPORT

A healthy 33-year-old woman, life-long nonsmoker, developed a persistent cough, progressive shortness of breath, and paroxysmal nocturnal dyspnea. Her previous medical history was unremarkable. She had no family history of breast cancer. After 1 week of symptoms, a chest x-ray was performed which revealed a partial right upper lobe atelectasis with an approximately 6 cm diameter, round, well-circumscribed lesion. On physical examination, she was dyspneic with a respiratory rate of 24 breaths/min and heart rate of 110 beats/min. Chest auscultation revealed inspiratory rhonchi and hypoventilation on the right. A chest computerized tomography revealed a large homogeneous mass with sharp borders and intraluminal extension in the right main bronchus (Fig. 1). Repeat chest x-ray at 24 hours revealed total collapse of the right lung. A flexible bronchoscopy revealed a polypoid lesion occluding the right main bronchus (Fig. 2). The lesion was firm, yellowish, and relatively avascular. Endobronchial biopsy specimens remained inconclusive. The clinical presentation suggested carcinoid tumor versus sarcoma. To have a better tissue sample, to assess preoperatively the extension of the lesion and to improve the respiratory function, a therapeutic bronchoscopy was performed and the tumor was resected using electrosurgery. Her symptoms improved considerably. Pathologic diagnosis of the tissue specimen demonstrated undifferentiated sarcoma. A right upper lobectomy was performed; the surgical specimen demonstrated a soft tissue mass, 6 cm in diameter. The histologic diagnosis was synovial sarcoma.

FIGURE 1.

FIGURE 1.

FIGURE 2.

FIGURE 2.

Physical examination revealed a hard lump in the right breast, 10×10 mm size, not attached to underlying structures. Contralateral breast also had a 15 mm size lump. The right lump had been detected previously during a gynecologic examination several months earlier and was felt to be a fibroadenoma and close follow-up was suggested. Both lesions were excised and the histopathologic examination disclosed synovial sarcoma in the left mammary gland and confirmed fibroadenoma on the right side. Immunochemistry of the lung and left breast lesion were identical. A whole body positron emission tomography scan was performed and did not show any other site of metastatic lesion.

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DISCUSSION

Metastatic tumors to the tracheobronchial tree are unusual, and occur in 2% of patients with solid tumors.1 The prevalence depends in fact on how the metastases are defined. If invasion of tracheobronchial structures by parenchymal or lymph node mass is included, the prevalence rate is higher. However, if they are defined as only direct metastases in the tracheobronchial tree from extrapulmonary lesions, the prevalence rate is much lower.8 The frequently associated primary tumors include those of the kidney, pancreas, colon, ovary, and thyroid.8 Even when the clinical and roentgenographic features of endobronchial metastasis and bronchogenic carcinoma are usually indistinguishable, in the majority of cases the primary tumor site is clinically apparent.1,9

Mammary carcinoma is likely to metastasize relatively early to the regional lymph nodes and thereafter primarily to the lungs, bone marrow, liver, and bone. Although pulmonary metastasis with invasion of a bronchus is reported, primary metastasis to an airway is distinctly rare.10 Whatever the histologic type, endobronchial metastasis tend to occur at a significant interval from the diagnosis of the primary tumor, indicating a relatively slow disease progression. A reported mean interval from the diagnosis of the primary carcinoma to the diagnosis of primary tracheobronchial tree involvement is between 59 months and 5.4 years9,11; however, the shortest reported time was 0 months in a patient with uterus carcinoma. In contrast to lung parenchymal metastases, the significantly lower number of reported tracheobronchial metastases is thought to be secondary to a difference in blood supply. The lung parenchyma is supplied by the pulmonary artery, through which all systemic blood passes; whereas, the trachea and bronchi are supplied by the bronchial artery receiving only a small portion of the systemic circulation. Thus, hematogenous spread is more likely to occur in the lung parenchyma.12

Synovial sarcoma is rare. It accounts for between 5% and 10% of the approximately 10,000 new soft tissue sarcomas reported each year.13 Most of the synovial sarcomas occur in the limbs. Primary pulmonary sarcoma has been described, but it is exceptional.14 In contrast, primary sarcoma occurring in breast is extremely rare and comprises 0.5% to 1% of all breast neoplasm. Only a few hundred cases of sarcomas other than cystosarcoma have been reported.15 In one of the few series of primary breast sarcomas, no single synovial sarcoma was observed in 42 cases.16 Because of the extremely unusual occurrence of this sarcoma in the breast, the possibility of a dual metastasis (lung and breast) or a primary lung sarcoma metastasizing in the breast was considered. The existence of a primary tumor in a more usual location seems to have been discarded by a negative positron emission tomography scan, which has shown to be extremely sensitive to detect sarcoma lesions.17 Extramammary malignancies metastasizing to the breast are also very rare and constitute approximately 2% of all breast tumors, but at least 1 case of synovial sarcoma with breast metastasis has been described.18

Our patient showed a combination of situations, which were each extremely unusual: a breast synovial sarcoma, an endobronchial metastasis of a soft-tissue sarcoma, and metastatic endobronchial disease as a first symptom of a malignant tumor. This exceptional scenario does not allow to accurately predict the survival expectancy. Unfortunately each one of these situations has a poor prognosis by itself. Primary sarcomas of the breast are aggressive tumors and the 3-year disease-free survival is approximately 39%.15 As only the margin of first surgery was found to be a significant predictor of survival, all attempts should be made to achieve a negative margin. In contrast, the presence of endobronchial metastasis also carries a short life expectancy. Survival after the diagnosis of endobronchial metastasis is poor with reported mean survival times between 12 and 15 months.9,11,19 However, as some studies have reported longer-term survival (21 to 32 mo), aggressive treatment should be considered.8,20

Clinical and epidemiologic features must be considered in the presence of an endobronchial lesion. Despite the unusual occurrence of EBM, primary lung cancer in a nonsmoking young woman is also very unusual.

Recently, molecular methods have been used for identification of specific mutations using a reverse transcription-polymerase chain reaction. These methods could be helpful to distinguish between pulmonary or extrapulmonary sarcomas, and to assess relationship with poor prognosis. However, it is still not available for clinical purposes.21

EBM are usually associated with a previously known primary tumor. In patients with endobronchial tumors, in contrast, metastatic soft-tissue sarcoma should be included in the differential diagnosis.

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ACKNOWLEGMENT

The authors thank Ms Paula De Bonis for her technical and secretarial assistance in preparing this manuscript.

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REFERENCES

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Keywords:

atelectasis; endobronchial electrosurgery; endobronchial metastases; interventional bronchoscopy; synovial sarcoma

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