Urologic Oncology: Seminars and Original Investigations 26 (2008) 291–294
Original article
Pseudosarcomatous fibromyxoid tumor of the bladder Jose A. Karam, M.D.a, Wareef Kabbani, M.D.b, Arthur I. Sagalowsky, M.D.a,* a b
Department of Urology, University of Texas Southwestern Medical Center at Dallas, Dallas, TX 75390 –9110, USA Department of Pathology, University of Texas Southwestern Medical Center at Dallas, Dallas, TX 75390 –9110, USA Received 22 November 2006; received in revised form 15 January 2007; accepted 18 January 2007
Abstract Pseudosarcomatous fibromyxoid tumors are uncommon lesions with uncertain pathogenesis. We present the case of a 36-year-old healthy patient who was evaluated for painless gross hematuria. Work-up revealed a bladder mass that was initially treated with transurethral resection that showed pseudosarcomatous fibromyxoid tumors. However, the mass recurred and was subsequently treated definitively with partial cystectomy with no recurrence at last follow-up. Partial cystectomy is a viable option in the treatment of recurrent symptomatic pseudosarcomatous fibromyxoid tumors of the bladder, when performed selectively. © 2008 Elsevier Inc. All rights reserved. Keywords: Bladder neoplasm; Fibromyxoid; Partial cystectomy
1. Introduction A 36-year-old healthy male presented to a urologist in January 2006 with the complaint of painless gross hematuria and passage of clots. He had no history of smoking, pelvic irradiation, previous malignancy, or occupational exposure to chemicals. He was in good general health and denied weight loss, fevers, or night sweats. Intravenous urogram showed normal upper tracts, prompt renal contrast uptake and drainage, and suggestion of a bladder mass. Abdominal computed tomography (CT) showed a large heterogeneous bladder mass with suggestion of bladder wall involvement, filling much of the bladder lumen (Fig. 1A). No hydronephrosis, lymphadenopathy, or metastases were noted. Cystoscopy revealed a large, nodular, white tumor covered by mucus, at the high posterior bladder wall. Partial transurethral resection of bladder tumor (TURBT) was then performed. Pathology revealed pseudosarcomatous fibromyxoid tumor with no evidence of malignancy. The patient was then referred to our center for further evaluation and treatment. At this point, the patient still had intermittent gross hematuria, some bladder pain, but no bowel complaints. In
* Corresponding author. Tel.: ⫹1-214-648-3976; fax: ⫹1-214-6488786. E-mail address:
[email protected] (A.I. Sagalowsky). 1078-1439/08/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.urolonc.2007.01.021
March 2006, a restaging TURBT was performed to evaluate further the extent of disease. Again, a large, white pedunculated mass in the high posterior wall was noted. Cystitis cystica was noted around this mass. Random cold cup biopsies were taken in anticipation for possible partial cystectomy at a subsequent stage. A lengthy, careful transurethral resection of this mass was then undertaken. Portions of this mass were quite firm, while other areas were velvety and thin. After resection of all visible tumor was accomplished, roller-ball electrocautery was used for hemostasis. At conclusion of the procedure, there was no visible perforation, no active bleeding, and no visible tumor. A catheter was then placed. Before resection, bimanual examination under anesthesia showed no evidence of gross bladder mass, and the bladder itself was mobile. Gravity cystogram was performed 5 days later, showed no perforation, and the catheter was removed. Final pathology again revealed pseudosarcomatous fibromyxoid tumor. Random bladder biopsies were consistent with benign bladder mucosa. Follow-up cystoscopy in May 2006 revealed only a small intraluminal nodule with innocent features at the dome of the bladder. However, CT of the abdomen and pelvis performed 10 days later showed a heterogeneous 8.8 ⫻ 9.3-cm heterogeneous mass at the bladder dome. Again, no hydronephrosis, lymphadenopathy, or metastases were noted. In June 2006, magnetic resonance imaging of the abdomen and pelvis showed a well-defined lobulated mass filling much of the bladder, still confined to the bladder, with multiple
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Fig. 1. CT of the abdomen and pelvis showing a large heterogeneous mass (A) occurring from the bladder midline and absence of the mass 3 months after partial cystectomy (B).
pseudoseptations, and a prominent postcontrast enhancement indicating a high degree of vascularity. No involvement of the urachus was noted on sagittal views. Because of the rapid recurrence of this tumor and the extent of bladder involvement, more radical surgical treatments were discussed with the patient, including partial cystectomy, radical cystectomy with prostate capsule sparing, and radical cystoprostatectomy with neobladder formation. The preoperative decision that was made with the patient was to perform a partial cystectomy, if technically achievable with good bladder preservation. Later in June 2006, the patient was taken to the operating room and placed supine on the operating table. After general anesthesia was achieved, a vertical incision was made, including an ellipse of skin around the umbilicus in preparation to remove the urachus en bloc with the tumor upon entering the abdomen. No visceral abnormalities or palpable adenopathy was noted upon inspection of the abdomen and pelvis. The bladder mass was easily felt. The lateral peritoneal wings of the bladder were incised, and the peritoneum was incised downwards until the vasa deferentia were reached and were preserved. The prevesical space was then developed anteriorly and laterally. At this point, this large mass could easily be grasped between the surgeon’s fingers and mobilized upward (Fig. 2), leaving an adequate amount of normal bladder tissue for reconstruction after partial cystectomy. A large atraumatic clamp was then placed underneath the mass (Figs. 3, 4). Flexible cystoscopy was then performed and showed absence of visible tumor beyond the clamp location. The bladder was opened distal to the clamp using electrocautery, and inspection showed normal trigone and ureteral orifices, with no residual visible tumor. Frozen section of the urothelial mucosal margins were negative for tumor. Frozen section of the mass was consistent with the previous pathology. Lymphadenectomy was not performed. The bladder remained satisfactory in size and
was closed in 2 layers after placement of a urethral catheter. A flat Jackson-Pratt drain was placed in the perivesical space. The abdomen was then closed in the standard fashion. The patient did well postoperatively, and the drain was removed before discharge home. Ten
Fig. 2. Intraoperative view of the bladder mass in between the medial umbilical ligaments. Normal bladder is not seen in this picture.
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Fig. 3. Sagittal cut of the bladder showing the urachus, a mass at the bladder dome, a ureteral orifice, and the prostate.
days later, a gravity cystogram showed no extravasation, and the catheter was then removed. Final pathology revealed pseudosarcomatous fibromyxoid tumor consistent with previous reports. The tumor measured 11 cm in its largest dimension (Fig. 5). The cut tumor had a multilobulated gelatinous appearance with lobules separated by multiple thickened fibrous bands. Histologically, the lesion had a pushing, well-circumscribed border, and consisted of alternating sparsely and moderately cellular areas showing proliferation of bland spindle and occasional stellate cells arranged in an interlacing storiform growth pattern. The nuclei were uniform to mildly pleomorphic, oval, and normochromatic with small visible nucleoli, and the cytoplasms were eosinophilic with tapered ends. Rare mitotic figures (⬍1/10 high-power field) with no significant atypia or necrosis were noted. The stroma showed prominent myxoid changes with slit-like vessels and mixed inflammatory infiltrates, mostly composed of lymphocytes with few plasma cells (Fig. 6). Mucosal margins were free of tumor. The tumor extended into the muscularis propria,
Fig. 4. Sagittal view of the bladder with 1 technique to perform partial cystectomy, here depicting the use of a scalpel to cut the bladder between 2 Glassman bowel clamps.
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Fig. 5. Ex vivo view of the opened partially resected bladder with the tumor inside. Gelatinous material was noted along with hard, white material.
but not into serosa or fat. In October 2006, CT of the abdomen and pelvis showed complete resolution of the bladder mass with no evidence of lymphadenopathy (Fig. 1B). At the most recent follow-up in November 2006, the patient did not have any gross hematuria, and only had mild frequency and urgency; urinalysis was negative for microscopic hematuria, and cystoscopy revealed no tumor recurrence within the bladder. The patient will undergo follow-up cystoscopy, and CT of the abdomen and pelvis in 2007.
2. Discussion Pseudosarcomatous fibromyxoid tumors are uncommon lesions with uncertain pathogenesis. The first description of such a lesion in the bladder was in 1980 when a spindle cell
Fig. 6. High-power magnification (magnification ⫻400) of a hematoxylineosin representative slide showing inflammatory cells and cellular atypia in a myxoid background consistent with pseudosarcomatous fibromyxoid tumor.
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lesion of the bladder was described as having a reactive pseudosarcomatous response [1]. Histologically similar lesions have been reported in the literature using different names, such as pseudosarcomatous fibromyxoid tumor, inflammatory pseudotumor, inflammatory myofibroblastic tumor, pseudosarcomatous myofibroblastic tumor, fibromyxoid pseudotumor, pseudomalignant spindle cell proliferation, and nodular fasciitis [2]. These lesions were subsequently described in the ureter [3], vagina [4], prostate [5], and urethra [5]. Recently, expression of anaplastic lymphoma kinase (Alk)-1 has been described in these tumors [6]. The Alk gene was originally identified in anaplastic large-cell lymphoma carrying the t(2;5)(p23;q35) translocation [7]. Most of the reported cases have occurred in young women. The differential diagnosis should include sarcoma (particularly low-grade leiomyosarcoma) because of the mesenchymal proliferation, but the lesion that was excised did not have any of the features consistent with sarcomas. Low-grade leiomyosarcoma shows a higher degree of hyperchromasia, focal coagulative necrosis, and increased mitoses, although few cases of pseudosarcomatous fibromyxoid tumor have been reported to be mitotically active. In rare cases, carcinomas of the bladder may have spindle-cell morphology (sarcomatoid carcinomas) and may occasionally be misinterpreted as a sarcoma. A potential pitfall considering this differential diagnosis is interpreting positive cytokeratin immunoreactivity as a definitive feature of sarcomatoid carcinoma because a relatively high percentage of pseudosarcomatous fibromyxoid tumors show Pan-cytokeratin reactivity [8]. A histologically similar entity to pseudosarcomatous fibromyxoid tumors is the postoperative spindle-cell nodule [9]. However, the postoperative spindle-cell nodule, in contrast to other spindle-cell proliferations of the bladder, has a unique relation to the recent performance of a bladder procedure, usually TURBT. It is disputed whether these entities are neoplastic or reactive because there is evidence to support both viewpoints. In our patient, pseudosarcomatous fibromyxoid tumor was diagnosed from the first resection, making the diagnosis of a postoperative spindle-cell nodule less likely. Other potentially helpful features that may assist in distinguishing between the pseudosarcomatous fibromyxoid tumor and postoperative spindle-cell nodule are the presence of foreign body giant cell granuloma and increased eosinophils in the latter. Both features were lacking in the
current case. Still, distinction between these 2 entities can be hard, if not impossible. It is unclear whether they represent the same entity. Harik et al. [2] recently studied 42 cases of pseudosarcomatous fibromyxoid tumor and postoperative spindle-cell nodule, and reported that the only differentiating factor is a history of TURBT in cases of postoperative spindle-cell nodule. They proposed that these 2 groups are similar enough to be considered the same entity, designated as “pseudosarcomatous myofibroblastic proliferation.” Because these tumors rarely recur locally or metastasize, a conservative approach is usually followed. In our patient, evidence of tumor was still present after 2 transurethral resections. At this point, it became evident that a more radical approach was needed. Fortunately for the patient, the mass was localized in the dome of the bladder and was successfully resected with partial cystectomy, without compromising the resection margins, and without the need for radical cystectomy or cystoprostatectomy and neobladder formation. References [1] Roth JA. Reactive pseudosarcomatous response in urinary bladder. Urology 1980;16:635–7. [2] Harik LR, Merino C, Coindre JM, et al. Pseudosarcomatous myofibroblastic proliferations of the bladder. A clinicopathologic study of 42 cases. Am J Surg Pathol 2006;30:787–94. [3] Horn LC, Reuter S, Biesold M. Inflammatory pseudotumor of the ureter and the urinary bladder. Pathol Res Pract 1997;193:607–12. [4] Guillou L, Costa J. Postoperative pseudosarcomas of the genitourinary tract. A diagnostic trap. Presentation of 4 cases of which 2 were studied immunohistochemically and review of the literature [in French]. Ann Pathol 1989;9:340 –5. [5] Young RH, Scully RE. Pseudosarcomatous lesions of the urinary bladder, prostate gland, and urethra. A report of three cases and review of the literature. Arch Pathol Lab Med 1987;111:354 – 8. [6] Tsuzuki T, Magi-Galluzzi C, Epstein JI. ALK-1 expression in inflammatory myofibroblastic tumor of the urinary bladder. Am J Surg Pathol 2004;28:1609 –14. [7] Morris SW, Kirstein MN, Valentine MB, et al. Fusion of a kinase gene, ALK, to a nucleolar protein gene, NPM, in non-Hodgkin’s lymphoma. Science 1994;263:1281– 4. [8] Iczkowski KA, Shanks JH, Gadaleanu V, et al. Inflammatory pseudotumor and sarcoma of urinary bladder: Differential diagnosis and outcome in thirty-eight spindle cell neoplasms. Mod Pathol 2001;14: 1043–51. [9] Proppe KH, Scully RE, Rosai J. Postoperative spindle cell nodules of genitourinary tract resembling sarcomas. A report of eight cases. Am J Surg Pathol 1984;8:101– 8.