A Single-Centre Experience on the Management of Adenosarcoma: A Successful Report of an Integrated Medical and Surgical Approach

Abstract

Adenosarcomas are the rarest form of uterine sarcomas, and clinical experience with their management is still limited. Here, we reported 7 patients with uterine adenosarcoma referred to our institution, focusing on main pathologic features, their medical history, and long-term follow-up. Among these patients, we provided a detailed description of the medical history of a 49-year-old woman with advanced uterine adenosarcoma with sarcomatous overgrowth who presented a brilliant radiologic and pathologic response after 3 cycles of epirubicin and ifosfamide, ultimately achieving an extraordinary long-term outcome through an integrated surgical and medical approach. Our single-centre experience would suggest that aggressive uterine adenosarcomas with sarcomatous overgrowth are sensitive to standard epirubicin and ifosfamide and that an integrated approach, both medical and surgical, could be considered in clinical practice, again emphasizing the relevant role of multidisciplinary management for this extremely rare disease.

Keywords: Adenosarcoma; biphasic tumours; endometriosis; epirubicin and ifosfamide; multidisciplinary management; sarcomatous overgrowth.

Figure 1.

(A) Coronal sequence, (B) axial sequence, (C) lateral sequence (November 2013): A computed tomographic scan revealed a huge pelvic mass of 14 cm × 10 cm × 13 cm with prominent colliquative aspects and heterogeneous contrast enhancement uptake.

Figure 2.

(A) Adenosarcoma with periglandular stromal condensation (Hematoxylin and Eosin, 100 x), (B) sarcomatous overgrowth with significant stromal pleomorphism (Hematoxylin and Eosin, 400 x), (C) the tumour cells show a heterogeneous immunoreactivity for CD10 (100 x), and (D) diffuse positivity for oestrogen receptor (100 x).

Figure 3.

December 2013: A post-operative computed tomographic scan revealed multiple peritoneal implants, confluent in a lobated mass with colliquative aspects.

Figure 4.

(A) Axial sequence, (B) coronal sequence (December 2013): A computed tomographic scan evaluation after 1 cycle of epirubicin and ifosfamide, performed earlier due to sudden onset of abdominal pain, showed a prominent increase in the pelvic mass (16 cm × 10 cm × 6 cm in diameter), with intra-lesional heterogeneous colliquative features and irregular and thick septa.

Figure 5.

January 2014: A computed tomographic scan evaluation after 3 cycles of epirubicin and ifosfamide showed a marked shrinkage of the pelvic mass (8.6 cm × 3.3 cm × 3.3 cm in diameter), largely necrotic and with heterogeneous aspects (axial sequence).

Figure 6.

(A) Axial sequence, (B) lateral sequence (March 2015): During a regular surveillance programme, a computed tomographic scan evaluation showed a pelvic solid mass, 6 cm × 3 cm × 4 cm of diameter, suggestive of relapse.

Figure 7.

(A) March 2015: ileal relapse of high-grade sarcomatous component of adenosarcoma with high mitotic count (Hematoxylin and Eosin, 50 x), (B) magnification of the high mitotic count highlighted by black arrows (Hematoxylin and Eosin, 400 x).