Page 74 - ECBIP 2021_Program
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6th European Congress ECBIP 15 - 17
  Book of Abstracts
for Bronchology and
Interventional Pulmonology ATHENS - GREECE 2021
OCTOBER
 MODERATED e-POSTER SESSION 07:
Lung Cancer
        PRIMARY MALIGNANT MELANOMA PRESENTING AS SUPERIOR VENA CAVA SYNDROME
Hanna Dawood1, Ludmila Guralnik2, Yaniv Zohar3, Elit Vainer Evgrafov1, Anna Solomonov1 1 Pulmonology Unit, Division of Internal Medicine, Rambam Health Care Campus, Haifa, Israel.
2 Medical Imaging Unit, Rambam Health Care Campus, Haifa, Israel.
3 Pathology Unit, Laboratory Services Division, Rambam Health Care Campus, Haifa, Israel
     PP45
    Introduction: Malignant melanomas account for 1.7% of all cancers. Primary malignant melanoma involving the mediastinum is very rare, and has only been documented in very few case reports (1-4). We describe a case of mediastinal primary malignant melanoma presenting as superior vena cava (SVC) syndrome.
Case Presentation: A 74-year-old woman with medical history of hypertension, presented to our emergency department with weight loss in the past 6 months and recent facial edema. She ruled out changes in her voice, dyspnea, fever or night sweats. The patient had history of 30-pack-year of smoking. Her physical examination revealed facial edema with no signs of distress. Chest CT scan (figure 1) showed a 4.6 X 5.4 X 12 cm heterogenous right mediastinal mass, invading the SVC and the lumen of right atrium. Lymphadenopathy in 2R, 4R, 7 (sub-carinal)
and 10R. Two lung masses: 3.1 x 3.2 x 3.5 cm in the right middle lobe and 2.7 cm in the left upper lobe, were noted. Bronchoscopy was performed and trans-bronchial needle aspiration from both the mediastinal mass and the sub- carinal lymph node was taken. Histologic diagnosis (figure 2) revealed malignant melanoma, confirmed by positive stain for SOX-10.BRAF was negative.KI-67 was 30%.The mass was not surgically resected due to its location and vascular invasion. Examination by a dermatologist and an ophthalmologist revealed no evidence of a primary skin or eye lesion.The patient received combined chemo and radiation therapy.
Discussion and conclusion: Malignant melanoma presenting as a mediastinal mass, without evidence of extra- thoracic disease is extremely rare with only a few cases reported (1,4).These tumors can be metastatic from primary cutaneous lesion that has regressed,or can also be de novo melanomas.To date,only three cases of SVC obstruction secondary to melanoma in the mediastinum have been reported in the literature (5-6). One was treated with BRAF inhibitor (7), another with chemotherapy and radiotherapy (6), while the third received endovascular stenting to the SVC (5).The prognosis,and management of primary malignant melanoma of mediastinum is unknown due to its rarity. Case reports suggest an aggressive clinical course. Radiotherapy is usually the first treatment of choice, and administered to control the local spread of tumor, particularly if the tumor is not amenable to surgery.
Acknowledgments: The authors thank the patient and her family.
Figure 1: Chest CT scan showing: heterogenous mediastinal mass (A) with superior vena cava and right atrium invasion (B), and abnormally enlarged sub-carinal lymph node (LN 7).
Figure 2: Sections from cell block showing atypical epithelioid cells and normal respiratory epithelium (H&E, 40x) (A). Atypical cells showing positive staining for SOX-10 (40x) (B).
  References:
1. Karuppiah SV, Buchan KG. Primary malignant melanoma: a rare cause of mediastinal mass. Jpn J Thorac Cardiovasc Surg 2006;54:396-398.
2. Lau CL, Bentley RC, Gockerman JP, Que LG, D’Amico TA. Malignant melanoma presenting as a mediastinal mass. Ann Thorac Surg 1999;67:851-852.
3. Pandya B, Ramraje S, Darade A. Primary malignant melanoma of the mediastinum. J Assoc Physicians India 2004;52:924- 925
4. Kim HR, Lee JS, Kim YT, Kim JH, Kim JE. Primary malignant melanoma presenting as an anterior mediastinal mass. Korean J Thorac Cardiovasc Surg 2002;35:697-700
5.Mónaco RG,Bertoni H,Pallota G,Lastiri R,Varela M,et al.2003.Use of self-expanding vascular endoprostheses in superior vena cava syndrome.Eur J Cardiothorac Surg 24(2): 208-211. https://doi.org/10.1016/s1010-7940(03)00293-8.
6. Mesko SM, Rosenthal KJ, Boasberg PD, Hamid O. 2015. BRAFtargeted therapy to treat superior vena cava syndrome in a patient with metastatic cancer. J Clin Oncol 33(25): e101-e103. https://doi.org/10.1200/jco.2013.49.5622.
7. Gaffey AC, Litzky LA, Sighal S. 2016. Primary mediastinal melanoma presenting as superior vena cava syndrome: A case study. Arch Clin Exp Surg 5(1): 56-58.
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