Japanese Journal of Clinical Oncology 30:37-39 (2000)
© 2000 Foundation for Promotion of Cancer Research
Pulmonary Lymphangitic Sarcomatosis from Cutaneous Angiosarcoma: an Unusual Presentation of Diffuse Interstitial Lung Disease
1Division of Hematology–Oncology, Department of Internal Medicine, 2Department of Pathology, 3Department of Diagnostic Radiology and 4Department of Orthopaedic Surgery, Chang Gung Memorial Hospital, Taipei, Taiwan
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ABSTRACT |
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 TOP ABSTRACT INTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
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Pulmonary lymphangitic sarcomatosis (PLS) is not much recognized clinically although it shows similar pathological patterns and diagnostic features to pulmonary lymphangitic carcinomatosis (PLC). We report a case with hand angiosarcoma whose chest X-ray findings revealed a diffuse interstitial pattern consistent with lymphangitic spreading. The final diagnosis was made by open lung biopsy. The clinical, diagnostic and pathological features of this disease process are reviewed.
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INTRODUCTION |
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TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
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The incidence of pulmonary lymphangitic carcinomatosis (PLC) accounts for only 6% of pulmonary metastases (1). Primary carcinomas such as breast, lung and stomach are the most common primary tumors reported to produce this form of dissemination (2). Lymphangitic lung metastasis from a sarcoma, presenting as a diffusely infiltrative lung disease, is rare. Only two previous reports have described two patients with primary pulmonary angiosarcoma (3) and intravascular bronchioalveolar tumors (IVBATs) (epithelioid hemangioendothelioma) (4) resembling lymphangitic carcinomatosis, respectively. Here, we present one case of pulmonary lymphangitic metastases from limb cutaneous angiosarcoma and describe the rare clinical manifestations. To the authors’ knowledge, this entity has not been reported previously.
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CASE REPORT |
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TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
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A 30-year-old female patient presented with multiple gradually enlarging, bruise-like papulonodular lesions on the skin of her left index finger over 2 years. On physical examination, these lesions were 0.5–3 cm in diameter, elastic to firm, brownish to tan and easily bled upon touching. Magnetic resonance imaging (MRI) showed multiple soft tissue masses over the radial dorsal side of the left hand. Subsequently she received a local excision of the tumor. Microscopic examination of resection demonstrated a grade III angiosarcoma with positive margin (Fig. 1). Immunohistochemical study revealed that the tumor cells were negative for factor 8 associated antigen. Local recurrence developed 3 months later, which led to a left below-elbow amputation in March 1996 and a computerized tomographic (CT) scan of the chest revealed no apparent metastasis. She was then followed up closely at our out-patient clinic with chest roentgenograms every 3 months.
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In June 1998, she complained of a productive cough with sticky whitish sputum, increasing dyspnea on exertion and intermittent low-grade febrile attack in the afternoon for a 1-month duration. On physical examination, the lungs were clear on auscultation; however, a chest X-ray revealed bilateral reticulonodular infiltrations involving both lungs without cardiomegaly or pleural effusion (Fig. 2a). High-resolution CT of the chest showed mediastinal and left hilar lymph node enlargement (Fig. 2b), and also thickening of the peribronchio-vascular interstitia, interlobular septa and interlobular fissures (Fig. 2c). Pulmonary tuberculosis and pulmonary lymphangitic metastases were considered first clinically. She denied exposure to tuberculosis. Laboratory data revealed a normal hematogram and biochemistry except for a leukocyte count of 2600 cells/mm3. Pulmonary function tests showed a forced expiratory volume at 1 s (FEV1) of 2.22 l/min (82% of predicted), a forced vital capacity (FVC) of 2.59 l/min (80% of predicted) and a diffusing capacity of the lungs for carbon monoxide (DLco) of 20.3 ml/min/mmHg (88% of predicted). No apparent evidence of extrathoracic disease was noted on further clinical evaluation except that a bone scan revealed a new lesion involving the left 10th rib on posterior aspect. Flexible bronchoscopy revealed normal tracheobronchial anatomy. We attempted a transbronchial biopsy but failed. Bronchoalveolar lavage (BAL) and the wash and brush specimens were negative for tumor and microorganisms (including bacterial, fungal and AFB). Consequently, she underwent an open biopsy in July 1998. The surgeon identified multiple small nodules and hypervascularity over the entire lung parenchyma. A wedge resection of the left lower lobe revealed tumor emboli in the vessels and lymphatic spaces and multiple nodules composed of pleomorphic spindle tumor cells (Fig. 3). Immunohistochemical study revealed that the tumor cells were negative for cytokeratin (AE1/AE3), HHF-35, S-100 and factor 8 associated antigen, but positive for vimentin and desmin; this was consistent with pulmonary lymphangitic sarcomatosis secondary to cutaneous angiosarcoma. Postoperatively, the patient refused further treatment and deteriorated rapidly. She died of respiratory failure on October 1, 1998.
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DISCUSSION |
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TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
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Angiosarcoma of the skin is a rare but highly malignant soft tissue tumor. Previous reports of angiosarcoma involving the lung have been largely case reports, with the majority describing metastatic disease to the lung. Metastases to the lungs occur in 64–79% of cutaneous angiosarcomas. Chest radiographs often disclosed multiple nodules. Pneumonia, cavitation of the lesions, atelectasis, pneumothorax and hemothorax have been reported (5). To the best of our knowledge, there have been no literature reports of pulmonary lymphangitic metastases from a sarcoma, thereby making this an extremely rare manifestation of sarcomas.
The most common pathway for pulmonary metastasis of sarcoma is via hematogenous dissemination and tumor embolization. Lymphangitic spread characterized by diffuse permeation of the pulmonary lymphatics is a late and distressing manifestation of malignancy that presages early death. This form of dissemination has been reported in a great variety of primary carcinomas. In contrast to carcinomas, sarcomas that metastasize to lymph nodes account for only 2–5% and primary sarcomas have not been recognized to exhibit lymphangitic spread in the lung. Thus metastatic sarcoma of the lung usually present themselves as multiple tumor nodules rather than interstitial infiltration. The clinical presentation of our patient was similar to that previously reported for patients with pulmonary lymphangitic carcinomatosis (PLC). Therefore, we have termed this type of metastasis ‘pulmonary lymphangitic sarcomatosis (PLS)’ to describe the rare clinical manifestation.
Because of the rarity of this disorder and its tendency to mimic other interstitial lung diseases, a diagnosis is often difficult to make and therefore delayed. The clinical characteristics of PLS are similar to those of PLC (6) and were exemplified in our patient. CT scan of the chest is more sensitive than plain radiography in the detection of lymphangitic spread. High-resolution CT scans show uneven thickening of the interlobular septa and bronchovascular bundles caused by combination of tumor cells in the lymph vessels, dilated lymphatics and desmoplastic reaction within these structures. The diagnosis must be confirmed by histological examination of the involved lung, which can be obtained by transbronchial biopsy or open lung biopsy.
In conclusion, we have reported a rare case of PLS from a patient with limb cutaneous angiosarcoma. The clinical pictures were a typical presentation of an interstitial pulmonary disease in terms of symptoms and radiographic examinations. The final diagnosis was made by an open lung biopsy. Although it may be the first reported case of PLS, for sarcoma patients with interstitial lung disease, PLS should be included in differential diagnosis.
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FOOTNOTES |
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+ For reprints and all correspondence: Chi-Ting Liau, Division of Hematology–Oncology, Department of Internal Medicine, Chang Gung Memorial Hospital, 199 Tung Hwa North Road, Taipei, Taiwan. E-mail: gerson@ms2.hinet.netAbbreviations: PLC, pulmonary lymphangitic carcinomatosis; PLS, pulmonary lymphangitic sarcomatosis; IVBATs, intravascular bronchioalveolar tumors; MRI, magnetic resonance imaging; CT, computerized tomography; FEV1, forced expiratory volume at 1 s; FVC, forced vital capacity; DLco, diffusing capacity of the lungs for carbon monoxide; BAL, bronchoalveolar lavage
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REFERENCES |
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TOPABSTRACTINTRODUCTIONCASE REPORTDISCUSSIONREFERENCES |
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1 Zieske LA, Myers EN, Brown BM. Pulmonary lymphangitic carcinomatosis from hypopharyngeal adenosquamous carcinoma. Head Neck Surg 1988;10:195–8.[Medline]
2 Coppage L, Shaw C, Curtis AM. Metastatic disease to the chest in patients with extrathoracic malignancy. J Thorac Imag 1987;2:24–37.[Medline]
3 Yousem SA. Angiosarcoma presenting in the lung. Arch Pathol Lab Med 1986;110:112–5.[Medline]
4 Yousem SA, Hochholzer L. Unusual thoracic manifestations of epithelioid hemangioendothelioma. Arch Pathol Lab Med 1987;111:459–63.[Web of Science][Medline]
5 Patel AM, Ryu JH. Angiosarcoma in the lung. Chest 1993;103:1531–5.
6 Trapnell DH. Radiological appearances of lymphangitis carcinomatosa of the lung. Thorax 1964;19:251–60.
Received July 7, 1999; accepted October 18, 1999.
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