HEAD ABOVE WATER: A RARE CASE OF SCALP ANGIOSARCOMA YIELDING SPONTANEOUS HYDROPNEUMOTHORAX

Abstract

TOPIC: Pulmonary Manifestations of Systemic Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Angiosarcomas are rare, highly aggressive neoplasms originating from vascular or lymphatic endothelium [1]. Cutaneous angiosarcoma of the scalp has a predilection to involve pleural/subpleural surfaces [2,3]. Able to induce pneumothorax, hemothorax, and pleural effusion, few reports link angiosarcoma of the scalp to cystic lung disease [3]. Herein, we present a case of cutaneous angiosarcoma of the scalp associated with cystic lung disease and hydropneumothorax. CASE PRESENTATION: An 80-year-old Asian man with a pertinent medical history of smoking, atrial fibrillation, hypertension, and coronary artery disease presented with 2 weeks of dyspnea and cough. Additionally, he endorsed the worsening of a large fungating scalp lesion for the past 3 months. Biopsy of the lesion was diagnostic of well-differentiated and atypical cell angiosarcoma of the scalp. This admission, CXR showed left-sided, moderate hydropneumothorax. CT thorax revealed new cystic/cavitary disease bilaterally, not present on prior imaging. The patient was administered 100% FiO2 via NRB with no resolution of pneumothorax. Subsequent thoracocentesis produced exudate. Upon worsening of symptoms, bronchoscopy with biopsy was performed, and chest tube placement drained voluminous serosanguinous fluid. BAL, quantiferon gold, AFBx2, fungal culture, and ova/parasite all returned negative. No evidence of metastatic disease resulted from biopsy. Upon patient stabilization and without significant reaccumulation of fluid/hydropneumothorax, he was discharged. Follow-up with pulmonology/CVT was recommended for small-bore catheter placement and possible pleurodesis. Eventually, pleural fluid analysis identified the presence of nontuberculous mycobacteria. DISCUSSION: Constituting only 2% of all soft tissue sarcomas, angiosarcoma has a propensity to metastasize to the lung [2]. Our patient presented with cystic lung disease and spontaneous hydropneumothorax with no evidence of metastases from the primary site. A series of Japanese autopsy data compared scalp vs. non-scalp angiosarcoma, in which pneumothorax was solely observed in patients with the scalp subtype [4]. Only 24 cases of angiosarcoma with spontaneous pneumothorax have been reported from 1978-2014, with fewer cases documented since [5]. Limited reports have linked angiosarcoma to spontaneous hydropneumothorax, as seen in our case. CONCLUSIONS: Angiosarcoma of the scalp has a predilection to yield pulmonary sequelae. Considering the scarcity of documented cases, such diagnosis should be considered in patients presenting with cystic lung disease or hydropneumothorax. The eventual identification of concurrent nontuberculous mycobacterium infection within our patient is remarkable. Mycobacteria actuating cystic lung disease is unorthodox [6]. However, the distinct etiology of our patient's presentation may have been multifactorial in nature, compounding the originality of this case. REFERENCE #1: Somasekharan Nair KK, Zabell AS, Vo KL, Shaikh MA. Pneumothorax: a classical presentation of metastatic scalp angiosarcoma. Ann Thorac Surg. 2012 Sep;94(3):e77-8. doi: 10.1016/j.athoracsur.2012.02.030. PMID: 22916786.2. Andersen KF, Albrecht-Beste E, Berthelsen AK, Loft A. Angiosarcoma of the Scalp: Metastatic Pulmonary Cystic Lesions Initially Misinterpreted as Benign Findings on 18F-FDG PET/CT. Diagnostics (Basel). 2015 Dec 22;6(1):1. doi: 10.3390/diagnostics6010001. PMID: 26838798; PMCID: PMC4808816. REFERENCE #2: Olsen D, Molloy C, Sriram PS. Rapidly progressive lung cysts and pleural effusion: a case report. Case Rep Pulmonol. 2011;2011:790274. doi: 10.1155/2011/790274. Epub 2011 Sep 8. PMID: 22937430; PMCID: PMC3420654.4. Kitagawa M, Tanaka I, Takemura T, Matsubara O, Kasuga T. Angiosarcoma of the scalp: report of two cases with fatal pulmonary complications and a review of Japanese autopsy registry data. Virchows Arch A Pathol Anat Histopathol. 1987;412(1):83-7. doi: 10.1007/BF00750735. PMID: 3120405. REFERENCE #3: Chang JH, Kim JH, Hong SH, Song ME, Ryu YJ, Lee JH, Shim SS, Cho MS, Sim YS. Angiosarcoma presenting with spontaneous hydropneumothorax: report of a case and review of the literature. Open Respir Med J. 2014 Dec 26;8:48-54. doi: 10.2174/1874306401408010048. PMID: 25614772; PMCID: PMC4296473.6. Baldi BG, Carvalho CRR, Dias OM, Marchiori E, Hochhegger B. Diffuse cystic lung diseases: differential diagnosis. J Bras Pneumol. 2017 Mar-Apr;43(2):140-149. doi: 10.1590/S1806-37562016000000341. PMID: 28538782; PMCID: PMC5474378. DISCLOSURES: No relevant relationships by Nicholas Barreras, source=Web Response No relevant relationships by Azim Hemani, source=Web Response No relevant relationships by Michael Markos, source=Web Response No relevant relationships by Travis Yamanaka, source=Web Response