Complete remission of recurrent uterine angiosarcoma following long-term systemic chemotherapy with paclitaxel and carboplatin: A case report

Abstract

Angiosarcoma (AS) is a rare and aggressive malignancy of vascular endothelial origin, accounting for 2-5% of all soft tissue sarcomas. Primary uterine AS is extremely rare, with only ~30 cases reported in English literature. Its prognosis remains poor, with a 5-year survival rate of <35%, and there is currently no standard systemic chemotherapy established for unresectable or recurrent cases. This study presents the case of a 57-year-old woman with a 27×20 cm uterine mass causing severe anemia (hemoglobin 4.5 g/dl). Following total hysterectomy and bilateral salpingo-oophorectomy, histopathological analysis confirmed primary uterine AS (T1NXM0). Despite complete surgical resection, three new intra-abdominal tumors appeared within 1 month. The patient underwent 21 cycles of intravenous paclitaxel and carboplatin every 3-4 weeks, achieving complete disappearance of all metastatic lesions. The patient was still alive 31 months after the initial surgery without any sign of recurrence or metastasis. This case demonstrates the potential efficacy of paclitaxel and carboplatin combination chemotherapy for recurrent uterine AS, contributing valuable data to guide future therapeutic strategies for this rare malignancy with typically poor prognosis.

Keywords: angiosarcoma; carboplatin; chemotherapy; complete remission; metastasis; paclitaxel; recurrence; uterine angiosarcoma.

Figure 1.

Magnetic resonance imaging. (A) T2-weighted image. A 27×20 cm mass (white arrowhead) with heterogeneous high and low signal areas in the uterus. (B) Diffusion-weighted image. The solid part of the tumor showed a high signal (white arrowhead). (C) Apparent diffusion coefficient map. The solid part of the tumor showed a low signal (white arrowhead). (D) Sagittal section. There was no invasion into the cervix or malignant tumor of cervical origin (white arrowhead).

Figure 2.

Radiological findings. (A) Computed tomography scan. A heterogeneous, septate, mixed-density mass was seen in the transverse section. (B) Positron emission tomography-computed tomography. Hypermetabolism was seen in the area where the presence of a malignant tumor was suggested by magnetic resonance imaging (white arrowhead).

Figure 3.

Macroscopic image. (A) Intraoperative findings showed a smooth, enlarged uterus with numerous dilated blood vessels, and strong adhesion (white arrowhead) between the uterus and sigmoid colon. (B) A spongy, hemorrhagic tumor with necrotic tissue.

Figure 4.

Pathological findings. (A) Hematoxylin and eosin stain. The atypical cells with enlarged nuclei and distinct nucleoli formed a reticular structure like a vascular cavity and exhibited signs of proliferation. Magnification, ×100. (B) CD31 staining showed a strong positive signal in the malignant cells. Magnification, ×100. (C) CD34 staining showed a partially positive signal in the malignant cells. Magnification, ×200.

Figure 5.

Computed tomography scan performed 1 month after surgery. The largest of the three new solid tumors that had recurred in the abdominal cavity (maximum diameter 7 cm) (white arrowhead).