A case of a sporadic malignant peripheral nerve sheath tumor of the urinary bladder with concomitant in situ urothelial carcinoma treated by transuretheral resection

Abstract

Malignant peripheral nerve sheath tumor (MPNST) of the urinary bladder is a very rare clinical entity. The association of such a tumor with urothelial carcinoma is even more unusual. Differential diagnosis between coexisting two distinct primary tumors and carcinosarcoma of the urinary bladder is very important as both the treatment and prognosis vary widely. Herein, we report a case of an MPNST with a concomitant in situ urothelial carcinoma in a 53-year-old man. To our knowledge, this is the first documented case of MPNST of the bladder that is treated by transuretheral resection which is in contrast with the previous reports that used cystectomy.

Malignant peripheral nerve sheath tumour of the urinary bladder in a cat

“…VRN is a rare autosomal dominant disorder that is characterised by neurofibroma formation. It has been theorised that MPNST arises from bladder wall neurofibromas of nerve sheaths in autonomic nerve plexuses (Cordon‐Cardo 1997, Meuten 2002, Sari and others 2011). These bladder tumours carry a poor prognosis; they are locally invasive, may spread within the peritoneum and have been reported to cause distant metastases.…”

“…Isolated MPNST (MPNST in patients with no history of VRN) occurs most frequently in the retroperitoneum and lower extremities (Cordon‐Cardo 1997). Very few cases of isolated MPNST of the urinary bladder have been reported (Mimata and others 2000, Sari and others 2011).…”

“…In contrast, isolated MPNST in humans carries a poor prognosis similar to other high grade peripheral soft tissue sarcomas and are often locally invasive with distant metastases (Cordon‐Cardo 1997, Koestner and others 1999). Treatment of choice in both humans and animals is early radical tumour resection; other treatment modalities have been attempted but none has been proven superior (Schulman and others 2009, Sari and others 2011).…”

“…Other sites include the spinal canal (Okada and others 2007) and periocular tissues (Hoffman and others 2005) with reports of one perirenal mass (Stoica and others 2001, Schulman and others 2009). PNST has been reported in multiple species, and while it has rarely been reported to originate from the bladder wall in humans (Lott and others 2007, O’Brien and others 2008, Sari and others 2011) the urinary bladder has not been reported as a site of origin in domesticated animals.…”

BCG vaccine and pulmonary tuberculosis

“… Protection against leprosy [16][17]  Protection or delay in Buruli ulcer [18]  Immunotherapy of cancers such as bladder cancer [19], colorectal cancer [20], lung cancer [21] malignant peripheral nerve sheath tumor [22] and melanoma [23]  Diseases such as multiple sclerosis [24][25][26] and…”