Undifferentiated Pleomorphic Sarcoma of Liver: Case Report and Review of the Literature

Mass, Jirong Betty; Talmon, Geoffrey A.

doi:10.1155/2018/8031253

Cited by 8 publications

(4 citation statements)

References 30 publications

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“…Symptoms of primary hepatic UPS are generally nonspecific. Patients frequently complain of abdominal pain, weight loss, anorexia, fever, jaundice, malaise, and a palpable abdominal mass [ 4 , 5 ]. Diagnostic tools including radiological imaging and laboratory studies are insufficient to make the diagnosis of UPS.…”

Section: Discussionmentioning

confidence: 99%

Undifferentiated Hepatic Sarcoma: A Rare Case

Pellegrini¹,

Russe-Russe²,

Higgins³

et al. 2022

Cureus

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Primary hepatic undifferentiated pleomorphic sarcoma (UPS) is a rare malignant mesenchymal tumor with a nonspecific clinical and radiologic presentation. Primary hepatic UPS is often a diagnosis of exclusion made by multiple immunohistological testing that rules out hepatic, hematologic, neural, and epithelial origin. Stains for mesenchymal origin are usually the only positive stain and do not demonstrate evidence of specific mesenchymal cell differentiation. We report a case of a 56-year-old male with no significant past medical history that presented with complaint of epigastric abdominal pain of six months duration. A computed tomography (CT) scan of the abdomen and pelvis exhibited numerous hepatic masses involving right and left hepatic lobe. A CT-guided core needle biopsy discovered undifferentiated/pleomorphic sarcoma. Histomorphology showed spindle cell neoplasm without recognizable hepatic tissue. Immunohistochemistry (IHC) stains were positive for smooth muscle actin (SMA) but failed to establish a more specific histogenesis. Furthermore, IHC stains revealed spindle neoplastic cells with focal and patchy positive h-caldesmon (approximately 10-15% of neoplastic cells), and negative for desmin. Given these results, the diagnosis of undifferentiated/pleomorphic sarcoma was established. It is imperative to consider UPS in the differential diagnosis of large liver lesions without evidence of differentiation. Early identification of this rare tumor can prevent the possibility of distant metastasis and improve survival among patients.

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Section: Discussionmentioning

confidence: 99%

Undifferentiated Hepatic Sarcoma: A Rare Case

Pellegrini¹,

Russe-Russe²,

Higgins³

et al. 2022

Cureus

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“…The co-existence of a low attenuation solid mass and a cystic component with mural calcification may be an important hint to consider splenic UPS. Prior literature showed that UPS is FDG avid on FDG-PET/CT, [33–35] which appears to apply to splenic UPS as well. [27] CT findings as mentioned above may be useful to avoid delaying radical surgery early in the splenic UPS disease course.…”

Section: Discussionmentioning

confidence: 99%

Long-term disease-free survival of an undifferentiated pleomorphic sarcoma of the spleen: A case report and literature review

et al. 2022

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Introduction: Undifferentiated pleomorphic sarcoma (UPS) primarily occurs in the soft tissues of the extremities, trunk, and retroperitoneum. As the primary UPS of the spleen (splenic UPS) is extremely rare, to the best of our knowledge, only 19 cases have been reported in English literature. No cases of long-term survival without a local or distant recurrence have been reported. Patient concerns: We report the case of a 37-year-old man who was referred to our hospital for a splenic tumor. He had no past medical or relevant familial history. On abdominal computed tomography (CT), a low attenuation solid mass and cystic component with mural calcifications were present at the lower pole of his spleen. The fluorodeoxyglucose-positron emission tomography (CT) indicated it as malignant tumor of the spleen. Diagnoses: The patient’s provisional diagnosis was deduced to be angiosarcoma, which was the most common malignant tumor of the spleen. Interventions: An elective laparoscopic splenectomy was performed, and the histology of the tumor was consistent with UPS (pT1, pN0, cM0, and AJCC8th). No adjuvant therapy was administered. Outcomes: Ten years have passed since the patient’s splenectomy, and he continues to do well, without evidence of local or distant recurrence. Lessons: To the best of our knowledge, this is the first case of long-term recurrence-free survival after surgical management of a splenic UPS. It is probable that radical splenectomy during the disease played the most important role in the patient’s long-term survival. Understanding the characteristic findings of a splenic UPS in an abdominal CT may help to diagnose properly.

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“…In 2002, the World Health Organization (WHO) declassified MFH as a formal diagnostic entity and renamed it as UPS, not otherwise specified [2]. UPS represents a final common pathway in tumors that undergooes progression towards dedifferentiation [3]. We report a man with UPS presenting as an exophytic pedunculated tumor of the left scapula.…”

Section: Introductionmentioning

confidence: 99%