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Massive malignant solitary fibrous tumor arising from the bladder serosa: a case report.

Dozier J, Jameel Z, McCain DA, Hassoun P, Bamboat ZM - J Med Case Rep (2015)

Bottom Line: The inferior vena cava was compressed, and the mass extended inferiorly to his upper pelvis abutting the superior dome of his bladder.The histological features combined with immunophenotyping were suggestive of a malignant solitary fibrous tumor that grossly appeared to be growing from the bladder serosa, specifically the intraperitoneal superior dome of the bladder.Malignant solitary fibrosis tumors of the urinary bladder, however, are very rare.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Division of Surgical Oncology, Hackensack University Medical Center, 20 Prospect Avenue, Hackensack, NJ, 07601, USA. Dozierjm@njms.rutgers.edu.

ABSTRACT

Introduction: Solitary fibrous tumors are rare neoplasms of mesenchymal origin. They are often of low malignant potential and rarely metastasize. While they frequently arise from the pleura, they can occur at any soft tissue site in the body. We present a case of a large (28 × 21 cm) malignant solitary fibrous tumor arising from the bladder serosa. In addition, the clinicopathologic features, differential diagnosis, cytogenetics and management of this rare disease are discussed, along with a review of the existing literature on this topic.

Case presentation: An otherwise healthy 41-year-old Caucasian man presented with weight loss and progressive abdominal bloating. A subsequent computed tomography scan of his chest, abdomen and pelvis revealed a 26.8 × 21 cm intra-abdominal mass occupying most of his abdominal cavity. The inferior vena cava was compressed, and the mass extended inferiorly to his upper pelvis abutting the superior dome of his bladder. He underwent operative resection and the resected mass measured 28 × 21 × 18 cm and weighed 4.8 kg. The cut surface revealed a gray-white mass with an ill-defined whorled-like pattern, with randomly assorted tan fleshy nodules. A histologic evaluation revealed variable, alternating hypercellular and hypocellular areas, with areas of necrosis. The tumor cells varied from spindle to epithelioid within a hyalinized stroma. In the hypercellular areas, the tumor cells showed moderate atypia with high mitotic activity. The histological features combined with immunophenotyping were suggestive of a malignant solitary fibrous tumor that grossly appeared to be growing from the bladder serosa, specifically the intraperitoneal superior dome of the bladder. Our patient is currently eight months post-surgery without evidence of recurrence.

Conclusions: Extrapleural occurrences of solitary fibrosis tumors are being increasingly observed. Malignant solitary fibrosis tumors of the urinary bladder, however, are very rare. As there are no pathognomonic features of malignancy, surgical resection is often both diagnostic and therapeutic, as was the case in our report.

No MeSH data available.


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Bisected gross specimen revealing fleshy, tan nodules.
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Fig2: Bisected gross specimen revealing fleshy, tan nodules.

Mentions: On gross inspection, the mass measured 28 × 21 × 18cm and weighed 4.8kg. The cut surface revealed a gray-white mass with an ill-defined whorled-like pattern with randomly assorted tan fleshy nodules (Figure 2). A histologic evaluation revealed a microscopic margin negative resection (R0) with variable, alternating hypercellular and hypocellular areas, with areas of necrosis. The tumor cells varied from spindle to epithelioid within a hyalinized stroma (Figure 3a-b). In the hypercellular areas, the tumor cells showed moderate atypia with high mitotic activity (focally over 10 mitoses per 10 high power fields) and Ki67 positivity of 15% (Figure 3c). His immunohistochemistry analysis showed that the tumor cells were positive for vimentin, CD34 (Figure 3d), BCL-2 and beta-catenin, and negative for pan Cytokeratin, p63, Calretinin, SMA, desmin, S100, CD-31, CD-117, DOG1, EMA, STAT6, GRIA2 and WT-1. His p53 immuno-histochemical stain was weakly positive, with 30 to 40% positivity. His cytogenetics analysis revealed no chromosomal rearrangements at loci 12q13 or 18q11.2.Figure 2


Massive malignant solitary fibrous tumor arising from the bladder serosa: a case report.

Dozier J, Jameel Z, McCain DA, Hassoun P, Bamboat ZM - J Med Case Rep (2015)

Bisected gross specimen revealing fleshy, tan nodules.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4358716&req=5

Fig2: Bisected gross specimen revealing fleshy, tan nodules.
Mentions: On gross inspection, the mass measured 28 × 21 × 18cm and weighed 4.8kg. The cut surface revealed a gray-white mass with an ill-defined whorled-like pattern with randomly assorted tan fleshy nodules (Figure 2). A histologic evaluation revealed a microscopic margin negative resection (R0) with variable, alternating hypercellular and hypocellular areas, with areas of necrosis. The tumor cells varied from spindle to epithelioid within a hyalinized stroma (Figure 3a-b). In the hypercellular areas, the tumor cells showed moderate atypia with high mitotic activity (focally over 10 mitoses per 10 high power fields) and Ki67 positivity of 15% (Figure 3c). His immunohistochemistry analysis showed that the tumor cells were positive for vimentin, CD34 (Figure 3d), BCL-2 and beta-catenin, and negative for pan Cytokeratin, p63, Calretinin, SMA, desmin, S100, CD-31, CD-117, DOG1, EMA, STAT6, GRIA2 and WT-1. His p53 immuno-histochemical stain was weakly positive, with 30 to 40% positivity. His cytogenetics analysis revealed no chromosomal rearrangements at loci 12q13 or 18q11.2.Figure 2

Bottom Line: The inferior vena cava was compressed, and the mass extended inferiorly to his upper pelvis abutting the superior dome of his bladder.The histological features combined with immunophenotyping were suggestive of a malignant solitary fibrous tumor that grossly appeared to be growing from the bladder serosa, specifically the intraperitoneal superior dome of the bladder.Malignant solitary fibrosis tumors of the urinary bladder, however, are very rare.

View Article: PubMed Central - PubMed

Affiliation: Department of Surgery, Division of Surgical Oncology, Hackensack University Medical Center, 20 Prospect Avenue, Hackensack, NJ, 07601, USA. Dozierjm@njms.rutgers.edu.

ABSTRACT

Introduction: Solitary fibrous tumors are rare neoplasms of mesenchymal origin. They are often of low malignant potential and rarely metastasize. While they frequently arise from the pleura, they can occur at any soft tissue site in the body. We present a case of a large (28 × 21 cm) malignant solitary fibrous tumor arising from the bladder serosa. In addition, the clinicopathologic features, differential diagnosis, cytogenetics and management of this rare disease are discussed, along with a review of the existing literature on this topic.

Case presentation: An otherwise healthy 41-year-old Caucasian man presented with weight loss and progressive abdominal bloating. A subsequent computed tomography scan of his chest, abdomen and pelvis revealed a 26.8 × 21 cm intra-abdominal mass occupying most of his abdominal cavity. The inferior vena cava was compressed, and the mass extended inferiorly to his upper pelvis abutting the superior dome of his bladder. He underwent operative resection and the resected mass measured 28 × 21 × 18 cm and weighed 4.8 kg. The cut surface revealed a gray-white mass with an ill-defined whorled-like pattern, with randomly assorted tan fleshy nodules. A histologic evaluation revealed variable, alternating hypercellular and hypocellular areas, with areas of necrosis. The tumor cells varied from spindle to epithelioid within a hyalinized stroma. In the hypercellular areas, the tumor cells showed moderate atypia with high mitotic activity. The histological features combined with immunophenotyping were suggestive of a malignant solitary fibrous tumor that grossly appeared to be growing from the bladder serosa, specifically the intraperitoneal superior dome of the bladder. Our patient is currently eight months post-surgery without evidence of recurrence.

Conclusions: Extrapleural occurrences of solitary fibrosis tumors are being increasingly observed. Malignant solitary fibrosis tumors of the urinary bladder, however, are very rare. As there are no pathognomonic features of malignancy, surgical resection is often both diagnostic and therapeutic, as was the case in our report.

No MeSH data available.


Related in: MedlinePlus