Pathophysiology
+ general considerations
- Rhabdomyosarcoma is a very rare tumor which arises from pluripotential mesodermal cells of the urogenital ridge or similar cells in the Müllerian and Wolffian ducts during embryogenesis
- Rhabdomyosarcoma is characterized by local invasion with metastasis either rare or late in the course of disease
- Subtypes of rhabdomyosarcoma: embryonal, alveolar, and pleomorphic
- Subtypes are difficult to differentiate histologically and electron microscopy, immunohistochemistry (i.e., myoglobin and desmin) ± DNA ploidy may be required for definitive diagnosis
- Gross appearance: friable grape-like cluster or polypoid mass in the trigone or neck of the bladder causing urinary tract obstruction, hydronephrosis, and hydroureter
- Hypertrophic osteopathy without pulmonary metastasis is a common paraneoplastic syndrome
- Metastatic sites include mesentery, omentum, regional lymph nodes, adrenal gland, and liver
DIAGNOSIS
+ Signalment
- Young, large breed dogs < 2 years
- No sex predilection in dogs
- Breed predisposition: Saint Bernard
+ Clinical Signs
- Dysuria, hematuria, pollakiuria, and stranguria
- Vaginal discharge
- Urinary obstruction
- Incontinence
+ Urinalysis and Urine Sediment Cytology
- Hematuria and proteinuria are consistent findings on urinalysis due to ulceration of the urothelial mucosa
- Bladder wash cytology may improve diagnosis capabilities by reducing contaminants
+ Blood Tests
- Hematology and serum biochemistry findings are usually normal or non-specific
- Mild to moderate normochromic, normocytic anemia can be caused by either hematuria or bone marrow suppression secondary to chronic disease
- Uremia may result from neoplastic obstruction of urinary outflow or age-related renal failure
Imaging Studies
+ Survey Radiographs
- Imagining techniques include survey abdominal and thoracic radiographs, contrast radiography, and CT
- Survey radiographs: sublumbar lymph node enlargement, renomegaly, and metastatic disease in the pulmonary parenchyma or skeleton, particularly lumbar vertebrae and pelvis
- Positive contrast cystography is useful for identification of mucosal abnormalities and space occupying lesions
- Excretory urogram is indicated to determine the location and extent of obstructive urinary tract disease when the urethra cannot be catheterized
+ Ultrasonography
Ultrasonography is recommended to determine the location and extent of bladder involvement, regional lymph node size and appearance, and involvement of adjacent anatomical structures such as the colon
+ Biopsy
- Biopsy is required for definitive diagnosis of urinary tract tumors
- Biopsy techniques include FNA, needle biopsy, catheter biopsy, cystoscopic, and open surgery
- Percutaneous biopsy procedures are not recommended due to the risk of tumor seeding
- Catheter biopsy techniques correlate with surgical biopsy results in 73% of urethral and bladder tumors
- Inflammation secondary to necrosis and ulceration is common and may result in false-negative findings
+ Clinical Staging
Intergroup Rhabdomyosarcoma Study Committee Clinical Classification (postoperative staging system)
TREATMENT
+ Surgery
- Surgical techniques for management of bladder tumors include:
- Palliative procedures: tube cystostomy ± partial cystectomy
- Curative-intent procedures: partial cystectomy and total cystectomy with urinary diversion
- Cystostomy tube can be placed percutaneously or with either laparoscopic or open surgery
- Complications: stranguria, pollakiuria, hematuria, urine leakage around the stoma, and vesicoureteral reflux which predisposes to ascending UTI and tumor seeding of the upper urinary tract
+ Chemotherapy
- Rhabdomyosarcoma is treated with a combination of surgery, radiation therapy, and chemotherapy in humans
- Chemotherapy agents include vincristine, actinomycin-D, and cyclophosphamide
- Prolonged clinical remission has been reported in 1 dog treated with partial cystectomy, cyclophosphamide, and doxorubicin
- Partial response has been observed with vincristine, doxorubicin, and cyclophosphamide, but single agent doxorubicin was ineffective in another case
+ Prognosis
- Prognosis for dogs with rhabdomyosarcoma is poor due to infiltrative growth and urinary tract obstruction
- Multimodality treatment, especially chemotherapy, has improved the prognosis in children with rhabdomyosarcoma
- Prognostic factors in humans include histologic type and grade, DNA ploidy, and clinical staging
- Diploid embryonal rhabdomyosarcoma are more sensitive to chemotherapy and radiation therapy than aneuploid embryonal rhabdomyosarcoma