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Chronic Hemoptysis: Manifestation of a Rare, Obscure, and Fatal Diagnosis
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A4032 - Chronic Hemoptysis: Manifestation of a Rare, Obscure, and Fatal Diagnosis
Author Block: R. Klein1, V. Patel2; 1Internal Medicine, William Beaumont Hospital, Royal Oak, MI, United States, 2Pulmonary and Critical Care, William Beaumont Hospital, Royal Oak, MI, United States.
Introduction: Chronic hemoptysis can occur with several benign and ominous disease etiologies, yet can be difficult to distinguish from other sources of bleeding such as gastric or nasopharyngeal. We present a diagnostic challenge in a patient with pulmonary nodules and chronic hemoptysis.
Case Report: A 68 year-old female presented with a four month history of low volume hemoptysis and fatigue. Her past medical history included COPD, tobacco abuse, pulmonary nodules (stable since 2013), and a benign adrenal nodule. On presentation she was hypotensive, tachycardic, and initial hemoglobin was 5.4 g/dl. CT scan of chest, obtained secondary to hemoptysis, showed bibasilar patchy opacities. In addition, there was a 2-mm interval increase in one of the previously stable nodules and several new nodules. ANCA, Galactomannan, and respiratory cultures were negative. Due to significant drop in hemoglobin, upper endoscopy and flexible nasolaryngoscopy revealed no source of bleeding. Bronchoscopy showed no endobronchial lesions or active bleeding. Bronchoalveolar lavage and transbronchial biopsies showed no active infection or malignant cells. Transbronchial biopsy showed hemosiderin-laden macrophages suggestive of chronic hemoptysis. A PET-CT performed few months prior to hospitalization due to enlarging adrenal nodule had revealed no significant uptake. A CT guided core needle biopsy of enlarging pulmonary nodule showed malignant endothelial proliferation with high-grade nuclear atypia. The tumor cell immunophenotypes were consistent with endothelial differentiation suggestive of angiosarcoma (Positive CD31, ERG, CD34, and Factor VIII; Negative TTF1 and cytokeratin 7). The patient was referred to oncology for further treatment.
Discussion: Angiosarcoma is an extremely rare malignant subtype of mesenchymal tumors. The incidence is 2 cases per 1,000,000, with the most common presenting lesions found in skin, heart, liver, and breast. Metastatic and primary pulmonary lesions have been uncommonly reported. Chronic hemoptysis can be seen in infection, inflammation, or malignancy; although commonly attributed to benign etiologies. This case exemplifies the need to include pulmonary angiosarcoma as a differential in a patient presenting with pulmonary nodules and chronic low volume hemoptysis. Prognosis of pulmonary angiosarcoma is dismal and most patients succumb to death within months. Literature has reported surgical resection, radiation, and chemotherapy without significant success in most patients.
Conclusion: Pulmonary angiosarcoma is a very rare type of malignant vascular tumor without well-defined clinical features. Patients can present with chronic hemoptysis with multiple pulmonary nodules and/or consolidation. Outcome remains poor with currently available treatment options.
Author Block: R. Klein1, V. Patel2; 1Internal Medicine, William Beaumont Hospital, Royal Oak, MI, United States, 2Pulmonary and Critical Care, William Beaumont Hospital, Royal Oak, MI, United States.
Introduction: Chronic hemoptysis can occur with several benign and ominous disease etiologies, yet can be difficult to distinguish from other sources of bleeding such as gastric or nasopharyngeal. We present a diagnostic challenge in a patient with pulmonary nodules and chronic hemoptysis.
Case Report: A 68 year-old female presented with a four month history of low volume hemoptysis and fatigue. Her past medical history included COPD, tobacco abuse, pulmonary nodules (stable since 2013), and a benign adrenal nodule. On presentation she was hypotensive, tachycardic, and initial hemoglobin was 5.4 g/dl. CT scan of chest, obtained secondary to hemoptysis, showed bibasilar patchy opacities. In addition, there was a 2-mm interval increase in one of the previously stable nodules and several new nodules. ANCA, Galactomannan, and respiratory cultures were negative. Due to significant drop in hemoglobin, upper endoscopy and flexible nasolaryngoscopy revealed no source of bleeding. Bronchoscopy showed no endobronchial lesions or active bleeding. Bronchoalveolar lavage and transbronchial biopsies showed no active infection or malignant cells. Transbronchial biopsy showed hemosiderin-laden macrophages suggestive of chronic hemoptysis. A PET-CT performed few months prior to hospitalization due to enlarging adrenal nodule had revealed no significant uptake. A CT guided core needle biopsy of enlarging pulmonary nodule showed malignant endothelial proliferation with high-grade nuclear atypia. The tumor cell immunophenotypes were consistent with endothelial differentiation suggestive of angiosarcoma (Positive CD31, ERG, CD34, and Factor VIII; Negative TTF1 and cytokeratin 7). The patient was referred to oncology for further treatment.
Discussion: Angiosarcoma is an extremely rare malignant subtype of mesenchymal tumors. The incidence is 2 cases per 1,000,000, with the most common presenting lesions found in skin, heart, liver, and breast. Metastatic and primary pulmonary lesions have been uncommonly reported. Chronic hemoptysis can be seen in infection, inflammation, or malignancy; although commonly attributed to benign etiologies. This case exemplifies the need to include pulmonary angiosarcoma as a differential in a patient presenting with pulmonary nodules and chronic low volume hemoptysis. Prognosis of pulmonary angiosarcoma is dismal and most patients succumb to death within months. Literature has reported surgical resection, radiation, and chemotherapy without significant success in most patients.
Conclusion: Pulmonary angiosarcoma is a very rare type of malignant vascular tumor without well-defined clinical features. Patients can present with chronic hemoptysis with multiple pulmonary nodules and/or consolidation. Outcome remains poor with currently available treatment options.
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