Cardiac Masses - Cases

1357552 - An unusual cause of chest pain: A case of metastatic eccrine porocarcinoma to the right ventricle

Saturday, January 28, 2023

11:08 AM – 11:14 AM

Location: Sandpiper

Presenting Author(s)

MV

Mahesh K. Vidula, MD

Assistant Professor
University of Pennsylvania
Philadelphia, Pennsylvania, United States

Primary Author(s)

MV

Mahesh K. Vidula, MD

Assistant Professor
University of Pennsylvania
Philadelphia, Pennsylvania, United States

Co-Author(s)

BL

Benedicte Lefebvre

Assistant Professor
University of Pennsylvania, United States
RP

Raul Porto Perez

Fellow
University of Pennsylvania, United States
NU

Nnamdi Udeh

Resident
University of Pennsylvania, United States
NW

Nicholas Wilcox

Fellow
University of Pennsylvania, United States
ML

Michael Levin

Fellow
University of Pennsylvania, United States
TK

Taisei Kobayashi

Assistant Professor
University of Pennsylvania, United States
SJ

Saurabh Jha, MBBS MS

Associate Professor
University of Pennsylvania
PHILADELPHIA, Pennsylvania, United States
KD

Kevin Duffy

Associate Professor
University of Pennsylvania, United States
Victor A. Ferrari, MD

Chair, Penn Cardiovascular Imaging Council
Hospital of the University of Pennsylvania and Penn Cardiovascular Institute
Phila., Pennsylvania, United States
PB

Paco Bravo, MD

Assistant Professor
University of Pennsylvania, United States

Disclosure(s):

Saurabh Jha, MBBS MS: No financial relationships to disclose

Victor A. Ferrari, MD: No financial relationships to disclose

Description of Clinical Presentation: A 79-year-old man with a history of squamous cell carcinoma and eccrine porocarcinoma of the neck status post resection presented to the emergency department with chest pain. On examination, he was hemodynamically stable with a 2/6 holosystolic murmur audible at the left sternal border.

Diagnostic Techniques and Their Most Important Findings: Electrocardiogram showed normal sinus rhythm without ST-T wave abnormalities. Transthoracic echocardiography revealed a large mass in the right ventricle (RV) (Figure 1A). Cardiac magnetic resonance (CMR) demonstrated a 3.1 x 7.6 cm multilobulated mass in the RV that extended from the septum and into the RV outflow tract, but did not invade the septum or pericardium (Figure 1B). The mass was isointense on both T1-weighted and T2-weighted sequences, did not lose signal with fat saturation, and demonstrated heterogeneous first-pass perfusion and heterogeneous early and late gadolinium enhancement (Figure 2). These findings were suggestive of metastases to the RV with associated necrotic areas and possible thrombus. An oncologic ¹⁸F-FDG-PET/CT study fused with the CMR (Figure 3) revealed photopenic defects in the mass which correlated with the non-enhancing regions on CMR that were thought to be necrosis or thrombus, and an FDG-avid rim that correlated with the enhancing regions on CMR. The patient was not a candidate for surgical excisional biopsy due to thrombocytopenia. Echocardiography-guided biopsy of the mass showed metastatic eccrine porocarcinoma. The patient’s thrombocytopenia precluded chemotherapy, and the patient passed away weeks later due to complications from an ischemic stroke.

Learning Points from this Case: Eccrine porocarcinoma is a rare skin cancer arising from eccrine sweat glands, accounting for less than 0.01% of cutaneous neoplasms (1). The most common site of metastasis are regional lymph nodes, but other distant sites have been described, including bone, bladder, breast, lung, and liver (2,3). To the best of our knowledge, metastases of eccrine porocarcinoma to the heart have not yet been described. Surgical excision is recommended when feasible in locoregional disease, and chemotherapy/radiation is utilized in metastatic disease (2). CMR is an invaluable tool in the evaluation of cardiac masses, due to its ability to identify features characteristic of malignancy. In this case, the first-pass perfusion and early/delayed enhancement indicated vascularity, and the heterogenous nature of the enhancement suggested that the mass outgrew its blood supply, suggestive of malignancy. Correlation with FDG uptake on PET/CT increased the likelihood of malignancy, with confirmation provided by biopsy. Multimodality imaging and correlation with tissue is critical for accurate diagnosis of intracardiac masses and initiation of appropriate therapies.