Pseudosarcomatous myofibroblastic proliferations of the bladder

A clinicopathologic study of 42 cases

Lara R. Harik, Celine Merino, Jean Michel Coindre, Mahul Amin, Florence Pedeutour, Sharon W. Weiss

Research output: Contribution to journalArticle

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Abstract

Inflammatory pseudotumor or pseudosarcomatous fibromyxoid tumor and postoperative spindle cell nodule of the bladder are unusual lesions of uncertain pathogenesis which share overlapping, if not identical, histologic features. We present our experience with 42 cases, the largest series to date, to study the etio-pathogenesis, histologic features, biologic behavior and relationship to "inflammatory myofibroblastic tumor" of childhood. Patients ranged in age from 7 to 77 years (mean 47 y) and males predominated (3.2:1). Most patients presented with hematuria (31/42). Common associations were smoking (10/30) and previous instrumentation or surgery (9/42). The clinicopathologic features of patients having or not having prior instrumentation were identical. Grossly the lesions were polypoid or nodular and involved any portion of bladder wall, most commonly the dome (9/27) and measured 1 to 10 cm (mean 4 cm). They were composed of spindled and stellate cells arranged in a myxoid background with numerous inflammatory cells. Myxoid hypocellular areas were more pronounced near the mucosal surface with greater cellularity and a fascicular arrangement in the deep aspect of the lesion. "Atypical" features included mitotic activity (0 to 20/10 HPF; mean 2/10 HPF; median 1/10 HPF; none atypical), necrosis (22/42), and extension into muscularis propria (28/32) or perivesicular fat (3/8). Lesions were positive for cytokeratin (31/33), SMA (23/34), desmin (21/35), and Alk-1 protein (12/26). FISH confirmed the Alk-1 translocation in 4/6 cases. Treatment included transurethral resection (30/42), partial cystectomy (9/42), and total cystectomy (3/42). Initial diagnostic error resulted in radiotherapy and chemotherapy in 3 patients. Follow-up was available in 28 patients. (range 3to 93 mo; median 25 mo). Three patients developed recurrences, but none had metastases. Because the clinicopathologic features of lesions associated with and without instrumentation were similar and inseparable, we believe they are essentially the same entity, and propose the term pseudosarcomatous myofibroblastic proliferation. The preponderance of evidence which includes the extravesical growth, local recurrence, and Alk-1 gene translocation in some cases suggests perhaps a neoplastic process with limited growth potential. Even in the face of atypical histologic features (muscle invasion and necrosis) the prognosis is excellent. Despite the Alk-1 gene translocation, there continues to be sufficient evidence for regarding these as distinct from the so-called inflammatory myofibroblastic tumor of childhood.

Original languageEnglish (US)
Pages (from-to)787-794
Number of pages8
JournalAmerican Journal of Surgical Pathology
Volume30
Issue number7
DOIs
StatePublished - Jul 1 2006
Externally publishedYes

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Urinary Bladder
Cystectomy
Necrosis
Plasma Cell Granuloma
Neoplastic Processes
Recurrence
Neoplasms
Desmin
Hematuria
Growth
Keratins
Diagnostic Errors
Genes
Radiotherapy
Smoking
Fats
Neoplasm Metastasis
Drug Therapy
Muscles
Proteins

All Science Journal Classification (ASJC) codes

  • Anatomy
  • Surgery
  • Pathology and Forensic Medicine

Cite this

Pseudosarcomatous myofibroblastic proliferations of the bladder : A clinicopathologic study of 42 cases. / Harik, Lara R.; Merino, Celine; Coindre, Jean Michel; Amin, Mahul; Pedeutour, Florence; Weiss, Sharon W.

In: American Journal of Surgical Pathology, Vol. 30, No. 7, 01.07.2006, p. 787-794.

Research output: Contribution to journalArticle

Harik, Lara R. ; Merino, Celine ; Coindre, Jean Michel ; Amin, Mahul ; Pedeutour, Florence ; Weiss, Sharon W. / Pseudosarcomatous myofibroblastic proliferations of the bladder : A clinicopathologic study of 42 cases. In: American Journal of Surgical Pathology. 2006 ; Vol. 30, No. 7. pp. 787-794.
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