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.