Angiosarcoma in gastroduodenal tract before and after the treatment with pazopanib: a case report (with video)

Introduction

Angiosarcoma is a rare neoplasm that accounts for <2% of all sarcomas (which are rare malignancies themselves) and accounts for only 1% of solid organ tumors in adults.¹ Angiosarcomas can occur anywhere in the body but are most commonly found in the skin, subcutaneous tissues, breast, and heart. In the GI system, angiosarcoma typically develops in the spleen and liver; it is very rarely found in the stomach and small intestine.²

The clinical signs and symptoms of GI tract angiosarcoma are nonspecific, including nonspecific abdominal pain, weight loss, anemia, nausea/vomiting, intestinal obstruction, and hemorrhage.³ Here we present a rare case of metastatic angiosarcomas to the GI tract that improved with pazopanib treatment.

Case Presentation

A 55-year-old woman had a history of breast angiosarcomas with multiple metastases to the scalp, face, fingers, lungs, and liver (Fig. 1). She had symptoms of dizziness, shortness of breath, and intermittent passage of black stools for several weeks, which resulted from severe microcytic anemia (hemoglobin, 5.9 g/dL; mean corpuscular volume, 83 fl oz). An EGD was performed and revealed numerous round-shaped, central ulcerative lesions with black to purple discoloration over the gastric body and duodenal bulb (Fig. 2, Video 1 [available online at www.igiejournal.org]).

Figure 1.

Angiosarcoma lesions over the scalp, face, and fingers.

Figure 2.

Angiosarcoma lesions over the gastric body and duodenal bulb (before pazopanib treatment).

After 3 months of targeted therapy with parenteral pazopanib 800 mg/d, the patient’s GI bleeding and anemia improved significantly (hemoglobin, 12.5 g/dL). The gastroduodenal lesions also resolved after treatment with pazopanib for 3 months (Fig. 3) but recurred after 6 months of targeted therapy. Ultimately, the patient chose to have palliative care and died of sepsis and hemorrhagic shock.

Figure 3.

Angiosarcoma lesions resolved after treatment with pazopanib for 3 months.

Discussion

Angiosarcoma is a rare malignancy in overall cancer that is much less common in the GI tract. Angiosarcomas are aggressive mesenchymal sarcomas of cells with vascular endothelial features.^1,4 The etiology of angiosarcoma remains unclear, but it has been linked to a history of radiation, exposure to arsenic and vinyl chloride, chronic lymphedema, and certain genetic factors.⁵ Patients with GI angiosarcomas may present with nonspecific symptoms and bleeding episodes, either manifest or occult, as well as anemia due to the tendency of the lesions to bleed.^2,3

Due to the varied symptoms, inadequate specimen sampling, and histopathologic similarity of poorly differentiated carcinoma, it is difficult to make a definitive diagnosis of epithelioid angiosarcoma of the GI tract.⁶ Endoscopic mucosal biopsy samples with pathologic examination are typically used to diagnose mucosal GI angiosarcoma, but submucosal lesions may require an EUS to obtain tissue for pathologic diagnosis.⁷ GI angiosarcomas can manifest as flat, bright-red lesions, centrally ulcerated nodules, purpuric or hemorrhagic nodules, and submucosal masses.^2,4 For those unspecific features, differential diagnosis with other GI sarcomas or GI stromal tumors is required.

There are currently no standard guidelines for the treatment of GI angiosarcoma. Treatment options include surgical resection, radiation therapy, chemotherapy, and targeted therapy.^6,8 Chemotherapy and targeted therapy agents, such as docetaxel and/or paclitaxel, propranolol, bevacizumab, imatinib, and pazopanib, have been prescribed.⁸ The prognosis of GI angiosarcomas is very poor due to late diagnosis, resistance to current chemotherapies, bleeding tendency, and aggressive characteristics.^6,9 The majority of patients die within 6 to 12 months of being diagnosed.^9,10

Returning to our patient, significant therapeutic outcomes were observed with endoscopy after she had received pazopanib treatment for 3 months. However, there was a recurrence within a short span of 6 months. A previous phase III trial of pazopanib also reported a progression-free survival of 4.6 months compared with 1.6 months for placebo; the overall survival with pazopanib was 12.5 months, whereas it was 10.7 months with placebo.¹¹ These findings suggest that pazopanib, as an angiogenic inhibitor, may have a temporary tumor-shrinking effect on angiosarcoma. Moreover, using pazopanib as a treatment option for metastatic angiosarcoma could potentially prevent tumor progression, thus improving GI bleeding and quality of life for patients.

Conclusions

We report a rare case of angiosarcoma with GI involvement, displaying both gastric and intestinal features observed during an upper endoscopy. Moreover, pazopanib exhibits potential in providing temporary and substantial tumor reduction for metastatic soft tissue sarcoma, offering the possibility of improving GI bleeding and overall quality of life. It is important to note that the long-term prognosis for angiosarcoma is as severe as that of ordinary metastasis of soft tissue sarcoma.

Patient Consent

The authors have received appropriate patient consent for the publication of this article.

Disclosure

All authors disclosed no financial relationships.

Supplementary data

Video 1

Angiosarcoma lesions over the gastric body and duodenal bulb (before pazopanib treatment).