Characteristics of embryonal sarcoma of liver (ESL)
Embryonal sarcoma can manifest anywhere in the liver, but is more commonly seen in the right lobe. (8). Grossly, ESL appears as a sizable, solitary, and well-defined mass with variable hemorrhagic, necrotic, and cystic areas. Microscopically, the tumor exhibits loosely arranged medium to large spindles, pleomorphic cells with poorly defined borders, and giant cells showing severe atypia(9, 10).
ESL typically lacks an association with underlying liver diseases such as hepatitis and cirrhosis. Additionally, tumor markers such as AFP, CEA, and CA19-9 are usually not elevated(11).
Multimodal treatment strategies and outcomes in AESL
AESL has an aggressive clinical behavior but is potentially treatable with multimodality therapy including chemotherapy, surgery, radiation, and in select cases, orthotopic liver transplantation. Surgical resection alone results in poor outcomes, with an estimated overall survival of 37 percent (6). The treatment protocol typically involves neoadjuvant chemotherapy preceding liver resection, but in certain scenarios where hepatic resection is performed without prior chemotherapy, postoperative adjuvant chemotherapy with or without radiation serves as a viable alternative. (6, 12). In one study, this approach yielded promising survival rates at a median follow-up of 53 months. Remarkably, the result also included two individuals with high-risk AESL, one with metastatic disease and another with a ruptured tumor, both achieving first disease remission (12).
Management and Challenges of AESL During Pregnancy
Primary hepatic malignancies during pregnancy are exceptionally rare (13), and the occurrence of AESL in pregnant women is even more infrequent.
Cancer diagnosed during pregnancy necessitates interdisciplinary collaboration among medical specialties, considering the well-being of both the mother and the unborn child. The management of cancer in pregnancy lacked standardization, often required terminating the pregnancy in the first or second trimester and/or delaying oncological treatment after delivery(14, 15).
Concerns about administration of cytotoxic chemotherapy during pregnancy arise because chemotherapy preferentially kills rapidly proliferating cells, and the fetus represents a rapidly proliferating cell mass. Therefore, the optimal management strategy for AESL during pregnancy should be aimed at achieving the following objectives at the same time: Initiate postoperative chemotherapy as soon as possible, avoid fetal toxicity to chemotherapy agents, and avoid the risky very-low-birth weight delivery. Performing a malformation scan at weeks 18–20, assessing growth, amniotic fluid index as well as fetal doppler flows at weeks 22–24 are crucial steps to ensure pregnancy viability(16). In our case, fetal echocardiography revealed normal development, and the infant was delivered after it reached a birth weight of 1kg, which is comparatively less risky in low-birth-weight cases nowadays (17, 18). Effective antenatal treatment with steroids followed by successful post-delivery care for the preterm infant was the key to uneventful discharge of the preterm infant. The mother was also able to undergo adjuvant chemotherapy in relative healthy condition.
Literature review and positioning of our case within existing reports
To our knowledge, this case marks the seventh documented instance of AESL in a pregnant patient(19–24). In previously reported cases, two pregnant patients had undergone liver resection for AESL, with known recurrent and metastatic liver disease prior to conception. Tragically, the other two cases involved unresectable AESL diagnosed either during the late stages of pregnancy or shortly after delivery, leading to fatal outcomes. The fifth case presented a diagnostic challenge with a hemorrhagic mass, adding complexity to the differential diagnosis between hepatic adenoma and AESL. Notably, the last case underwent a single surgery that included essential procedures such as neonatal delivery, hysterectomy, and resection of the left liver lobe.
Among all the cases, our case appears to be the inaugural contribution in the literature to adopt a comprehensive and multidisciplinary approach involving various medical specialties. This distinctive case emphasizes a nuanced consideration of the intricate interplay between patient and fetus, taking into account factors such as radiation exposure, methods and maturity of delivery, as well as chemotherapy timing. The considerable length of the follow-up duration is also a significant highlight of this case.
Our integrated multidisciplinary treatment approach proved successful in this rare disease entity. The two-year follow up duration without recurrence is also a significant highlight of this case. Such favorable outcome of both the mother and child showed that AESL during pregnancy can be successfully treated. This case contributes valuable insights to the limited literature on AESL during pregnancy, showcasing the potential effectiveness of such multidisciplinary approach.