Ann Hepatobiliary Pancreat Surg.  2017 Nov;21(4):228-231. 10.14701/ahbps.2017.21.4.228.

Primary gigantic leiomyosarcoma of the liver treated with portal vein embolization and liver resection

• Giakoustidis D
• Giakoustidis A
• Goulopoulos T
• Arabatzi N
• Kainantidis A
• Zaraboukas T

• KMID: 2397804
• DOI: http://doi.org/10.14701/ahbps.2017.21.4.228

Abstract

Primary hepatic leiomyosarcoma (PHL) is an extremely rare tumor. The tumor has no specific presentations and often diagnosis is delayed until it reaches a significant size. We report the case of a 69-year-old female presented with a huge PHL. Due to size of the tumor and to be operable, the patient subjected to right portal vein embolization (PVE) and selective embolization of segment V. Four weeks after the PVE, liver resection was conducted (Segments V+VI bisegmentectomy plus resection of IVA). The patient had an uncomplicated post-operative course, and discharged at the 8th post-operative day. Diagnosis of PHL was confirmed by histopathological and immunohistochemical examinations. The patient refused to receive adjuvant chemotherapy, and revealed evidence of recurrence six months after the operation, and finally died 12 months after the operation and 16 months after initial diagnosis. PHL is an extremely rare tumor and often in first presentation has significant size. Radical surgery with adjuvant chemotherapy is key feature for prolonged survival.

Keyword

Primary hepatic leiomyosarcoma; Portal vein embolization; Liver resection

MeSH Terms

Aged
Chemotherapy, Adjuvant
Diagnosis
Female
Humans
Leiomyosarcoma*
Liver*
Portal Vein*
Recurrence

Figure

• 

  Fig. 1 (A and B) Abdominal computed tomography (CT) revealed a hypodense lesion (14 cm×11 cm) occupying segments IVA, V, VI and part 0f VII. (C) PVE of the right portal vein and selective embolization of the branch of segment IV was conducted. (D) Tumor measured 14 cm×11 cm prolapsing from the liver. (E) En-block resection of the omentum and the abdominal wall was conducted. Additionally, resection of segments IVA, V, VI, and part of VII were conducted with no need for Pringle maneuver.

• 

  Fig. 2 (A) L: Atypical spindle cells in fascicular arrangement (H+E, ×200). M: Area with large pleomorphic atypical cells (H+E, ×400). R: Right: Border of the tumor (down-left) and the hepatic parenchyma (up-right) (H+E, ×100). (B) The tumor cells are positive for immunostain SMA (immunostain, ×200).

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