This case study highlights the distinct challenges clinicians face when diagnosing and managing prostatic leiomyosarcomas and determining the etiology of concurrent urologic symptoms over the course of its management. Prostate leiomyosarcomas are extremely rare, as they compose 0.1% of primary prostate tumors; sarcomas account for 1% of malignancies, with 5% of sarcomas arising from the genitourinary tract [2]. In each of the rare cases, the condition may present with a plethora of non-specific genitourinary symptoms such as recurrent hematuria, urinary frequency or urgency, recurrent urinary tract infections (UTIs), urinary retention, or generalized suprapubic pain. The FNCLCC grading system and histopathological examination are typically used for the definitive diagnosis. The tumor histology is notable for marked necrosis, hypercellularity, nuclear pleomorphism without degenerative features, positive histochemical stains such as smooth muscle actin, Desmin, vimentin, calponin, and CD34, and is commonly differentiated from other prostatic stromal lesions known as STUMP [3]. In contrast to more common tumors of the prostate, normal or slightly elevated PSA values can be noted in prostate leiomyosarcomas, as the tumor has a nonepithelial origin. Although no standard recommendations are established to treat prostate leiomyosarcoma, a wide variety of treatments may be used and can include surgery, if operable, as well as chemotherapy and radiotherapy. However, the long-term survival rate and overall prognosis remains poor [4].
The described case features an unusual presentation of a rare prostate malignancy associated with multiple postoperative hospitalizations for recurrent hematuria, urinary retention, suprapubic pain, and urinary tract infections (UTIs). Although urinary frequency and urgency are commonly reported symptoms in patients with prostate leiomyosarcomas, recurrent hematuria and urinary retention have only been reported in a handful of isolated cases. In this case of a 61-year-old male with a strong smoking history and a normal PSA level, hematuria may be a rare obvious symptom that can delay its diagnosis [5]. This patient’s unique symptoms of recurrent hematuria and acute urinary retention may be associated with either the malignancy or due to a multitude of other factors.
The urinary retention and recurrent UTIs may have occurred secondary to a significantly enlarged prostate and underlying BPH. In contrast, the recurrent hematuria that began shortly after the prostatectomy may be attributed to a documented adverse event from either the operation [6], post-operational radiotherapy [7], or doxorubicin treatments [8]. Despite the multifactorial etiology of the recurrent hematuria, the hematuria was most likely caused directly by the leiomyosarcoma. This is because recurrent hematuria has been linked to prostate leiomyosarcomas due to hypervascularity [9, 10]. The involvement of the anterior transitional zone and right distal ureter in this patient’s tumor, rather than the commonly affected peripheral zone in other prostate malignancies, may have also contributed to the resulting hematuria. In addition, dysuria and stranguria, or symptoms of bladder outlet obstruction, have been noted to occur with prostate sarcomas [2, 4].
As prostate leiomyosarcomas with recurrent episodes of hematuria are rare, it is of note that this case may have an etiological genetic component. While the patient denied any family history of prostate cancer or bleeding disorders, the patient’s sister and maternal grandmother had breast cancer in their early 60s and 80s, respectively. In addition, his mother was diagnosed with pancreatic cancer at 75. This may be due to a germline mutation through his maternal lineage, as prostate cancers such as leiomyosarcomas have been associated with a family history of breast and pancreatic cancers diagnosed at a younger age [11].
Prostate leiomyosarcomas can have several systemic manifestations and may be difficult to diagnose in certain situations. Aside from the clinical symptoms due to the primary prostatic growth, the metastatic and extra-prostatic manifestations of the sarcoma, such as the ascites and osteoblastic pulmonary lesions, can be debilitating and have a significant impact on the quality-of-life of the affected patient. For this reason, its complex pathology requires clinicians to be aware of the masquerading effects of other genitourinary conditions and postoperative adverse events on potential neoplastic symptoms and the multifactorial etiology of non-specific symptoms such as hematuria and urinary retention.
The constellation of typical constitutional symptoms and physical exam findings, including recurrent hematuria, urinary retention, suprapubic pain, and UTIs, is non-specific and may be due to either infectious, iatrogenic, or neoplastic conditions. For this reason, determining the etiology of these symptoms is paramount in establishing a timely diagnosis and preventing significant delays in disease management and treatment. In addition, diagnostic tests such as a timely abdominal CT scan can detect malignant ascites that may significantly affect the patient’s prognosis and management. Although prostate leiomyosarcomas are not often seen in clinical practice, it is crucial to be aware of its presentation as an accurate diagnosis may improve the patient’s quality and life expectancy.