Relapsing uterine adenosarcoma presenting as pulmonary hypertension

• Type: Another Publication in an International Scientific Journal
• Date: 2022

Authors

Carreira, M (Author) Other; Lisboa-Goncalves, P (Author) Other; Matos, MI (Author) Other; Flores, L (Author) Other; Cunha, F (Author) Other; von Hafe P (Author) FMUP

Journal

Title: Gazzetta Medica Italiana Archivio per le Scienze Mediche

ISSN: 0393-3660

Publisher: EDIZIONI MINERVA MEDICA

Indexing

ISI Web of Knowledge - 0 Citations

Scopus

Other information

Authenticus ID: P-00X-GX3

DOI: 10.23736/s0393-3660.21.04574-5

Abstract (EN): Tumoral pulmonary hypertension encompasses pulmonary tumor microembolism and pulmonary tumor thrombotic mi-croangiopathy. It is usually associated with adenocarcinomas. We reported the case of a 60-year-old female patient pre-sented to our emergency department with progressive dyspnoea on exertion for one month. Two years prior to presenta-tion she had been diagnosed with a uterine adenosarcoma without sarcomatous overgrowth stage group IB and submitted to hysterectomy plus salpingo-oophorectomy. She was treated with anastrozole 1 mg id. Three months before admission, in a follow-up visit an asymptomatic elevation of alkaline phosphatase, gamma-glutamyl transferase and tumor marker CA 125 was apparent. No evidence of a primary or metastatic lesion was detected at the time. The patient reported no oth-er complaints, and her physical examination was unremarkable, except for peripheral arterial oxygen saturation of 86%, while the patient was breathing ambient air. Arterial blood gas analysis showed hypoxemia. A transthoracic echocardiog-raphy demonstrated an estimate pulmonary artery systolic pressure of 70 mmHg with right chambers enlargement. Right heart catheterization suggested precapillary pulmonary hypertension. She was started on ambrisentan 5 mg id, sildenafil 25 mg bid and anticoagulation. FDG-PET scan showed uptake in a very small fluid collection on the pouch of Douglas, which was impossible to drain by culdocenthesis. Pulmonary wedge aspiration cytology was negative for malignant cells. As the patient's clinical condition deteriorated with worsening hypoxia, right sided pleural effusion and aggravating cyto-cholestasis, she underwent a course of chemotherapy with doxorubicin even before histologic confirmation. A diagnostic thoracocentesis was performed which eventually confirmed metastatic adenosarcoma. After one cycle of chemotherapy the patient improved significantly with near normalization of liver biochemical tests. She was discharged after a second course of chemotherapy and remained well without any complications at two months of follow-up.

Language: English

Type (Professor's evaluation): Scientific

No. of pages: 6