Primary Esophageal Melanoma: A Case Report

6079 

Cardiothoracic Resident Case Report Competition 

Pooja Patel (1), Colton Boudreau (1), Samuel Jessula (2), Madelaine Plourde (1)

(1) Dalhousie University, Halifax, Nova Scotia, (2) Harvard University, Cambridge, MA

Pooja Patel    -  Contact Me
Dalhousie University

Colton Boudreau    -  Contact Me
Dalhousie University

Samuel Jessula    -  Contact Me
Harvard University

Madelaine Plourde    -  Contact Me
Dalhousie University

Pooja Patel    -  Contact Me
N/A

An 80-year-old female was referred to our thoracic surgery service with a one-year history of progressive dysphagia and ten-pound weight loss.

Low-dose CT imaging was obtained which noted a soft tissue mass, likely intraluminal, measuring 3.1 x 2.6 x 5.5 cm. Bronchoscopy revealed extrinsic compression, but no airway involvement, and EGD confirmed the presence of a large tumour spanning the proximal-esophagus from 21 to 29 cm from the incisors.

Multiple tissue biopsies were obtained during EGD, which demonstrated histological findings in keeping with melanoma. Pathology noted that this specimen may represent a primary esophageal malignant melanoma but could represent metastatic disease from skin or mucosal surfaces. As such, investigations to locate a primary source involved full skin surveillance exam, consultation with ophthalmology to rule out an ocular melanoma and a PET study.

Each of the aforementioned diagnostic management steps yielded normal results. The PET scan revealed an uptake in the left cavernous sinus; however, subsequent MRI of brain revealed no concerning focal lesions. Unexpectedly, despite attempts to locate a primary melanoma source, no such source could be found, leaving primary esophageal melanoma as the diagnosis.

Medical oncology considered targetable tumour markers from the detailed pathology assessment, but the tumour lacked mutated BRAF expression. The case was discussed at the multi-disciplinary tumor board. Systemic treatment options other than systemic immunotherapy in the adjuvant setting were not recommended. Radiation oncology indicated that their role would be limited to a palliative setting. Given the patient's limited comorbidities, the consensus was that an esophagectomy would be the preferred treatment modality.

The patient was agreeable to surgical resection and underwent a minimally invasive 3-hole esophagectomy with gastric pull-up and pyloromyotomy. The procedure was uncomplicated and the specimen along with station 7, 8 and 9R lymph nodes were sent for histological analysis.

On pathology, the tumor was located entirely within the tubular esophagus, not involving the gastroesophageal junction and measured 7.2 x 6.5 x 2.0 cm. No lymphovascular or perineural invasion was identified. All lymph nodes (0/8) and margins were uninvolved.

The patient's post-operative course in hospital was complicated by a short two-day intensive care unit stay where she required vasopressor support and supplemental oxygen. The remainder of her hospital stay was otherwise unremarkable.

Beyond her initial discharge from hospital, she has required upper endoscopies every one to two months for dilations of an anastomotic stricture 16 cm from the incisors that caused her dysphasia. It was felt that this was an ischemic stricture related to a proximal anastomosis.

18 months after surgery, the patient developed worsening headaches. This prompted a CT head which was negative. A CT chest/abdomen/pelvis was also completed to assess for recurrence and an irregularly shaped subsolid nodule in the right upper lobe measuring 16 x 11 x 15 mm was found. Mild uptake of this lesion was seen on PET scan. Core biopsy of the lesion revealed moderately differentiated adenocarcinoma, KRAS positive and PD-L1 negative. She was treated with 3400 cGy in a single fraction by the Radiation Oncology team.

The patient is now 26 months from surgery without evidence of melanoma recurrence.

Thoracic