Associazione Nazionale Medici Cardiologi Ospedalieri



Mediastinal round cell sarcoma with pulmonary artery stenosis: Clinical usefulness of cardiac MRI in Cardio-Oncology

Canale Maria Laura Lido Di Camaiore (Lu) – Cardiologia, Ospedale Versilia – A Nome Dell’Area Di Cardioncologia- | Bisceglia Irma Roma (Roma) – Servizi Integrati Di Cardiologia, Azienda Ospedaliera San Camillo Forlanini | Gallucci Giuseppina Rionero In Vulture (Potenza) – Unità Di Cardioncologia- Irccs-Crob | Turazza Fabio Maria Milano (Milano) – Unità Di Cardiologia, Fondazione Irccs Istituto Nazionale Dei Tumori | Camilli Massimiliano Roma (Roma) – Dipartimento Di Medicina Cardiovascolare, Fondazione Policlinico Universitario A. Gemelli Irccs | Russo Giulia Trieste (Trieste) – Azienda Sanitaria Universitaria Giuliano Isontina (Asugi) | Lestuzzi Chiara Aviano (Pordenone) – Unità Di Cardiologia, Dipartimento Di Oncologia Cro Istituto Nazionale Tumori | Paccone Andrea Napoli (Napoli) – Dipartimento Di Cardiologia, G. Pascale Fondazione Istituto Nazionale Tumori (Irccs) | Fiscella Damiana Catania (Catania) – U.O.C. Cardiologia, Ospedale Garibaldi-Nesima, Azienda Di Rilievo Nazionale E Alta Specializzazione “Garibaldi” | Maurea Nicola Napoli (Napoli) – Dipartimento Di Cardiologia, G. Pascale Fondazione Istituto Nazionale Tumori (Irccs) | Bucciarelli Ducci Chiara London (London) – Royal Brompton & Harefield Hospital King’S College | Baldi Giacomo Prato (Prato) – Oncologia Medica Ospedale Santo Stefano | Casolo Giancarlo Lido Di Camaiore (Lucca) – Cardiologia – Ospedale Versilia | Camerini Andrea Lido Di Camaiore (Lucca) – Oncologia Medica Ospedale Versilia

Case description: a previously healthy 26-year-old man presented with dyspnea on exertion and dry cough. CT scan revealed a large mediastinal mass with displacement of great vessels and trachea and pericardial effusion. Cardiac MRI showed the huge mediastinal mass, literally leaning on the heart with signs of compression of the pulmonary artery (Figure 1) with the typical D-shape of inter ventricular septum. Pericardial effusion did not compress the RV due to high intraventricular pressure. At baseline echo the mass simulated pulmonary artery stenosis as a consequence of pulmonary artery “ab extrinseco” compression. Surgical biopsy showed Ewing sarcoma lately redefined into undifferentiated round cell sarcoma so the patient started chemotherapy with VAI (vincristine, adriblastine, and ifosfamide) x6 followed by maintenance etoposide and ifosfamide (no anthraciclines for risk of cardiac toxicity). After induction phase MRI showed a partial response to treatment; mediastinal mass further reduced at the end of maintenance (Figure 2). Pericardial effusion disappeared and peak velocity of pulmonary artery went back to normal level at echo.

Our patient underwent surgery with en-block removal of mediastinal mass with pericardium and anonymous vein and partial pulmonary upper left lobe resection with R0 resection. Pathology report confirmed an undifferentiated round cell sarcoma (possible embryonal origin, FISH analysis for EWS/FUS genes and 12p negative). Adjuvant mediastinal radiotherapy was delivered. The patient is alive without disease recurrence at one-year follow-up.

Conclusions: cardiac MR offer great tissue characterization (differential diagnosis between malignant and benign masses) inside/outside the heart. CMR is non-invasive/non radiation and ideal technique for surgery indication and follow-up imaging.