permatic cord sarcomas are rare tumors of the paratesticular region affecting primarily older men. Because
these tumors are not well studied, their incidence has not been
quantified. Patients typically present with a nontender mass
within the inguinal region or scrotum, sometimes confused
with either an inguinal hernia or a testicular tumor. Without
complete and appropriate imaging and diagnostic studies, most
of these patients are taken to the operating room for herniorrhaphy or exploratory surgery, with a cancer diagnosis made
intraoperatively. In addition, there is no current well-defined
standard for radiographic evaluation of the inguinal region.
Ultrasonography is indicated for testicular and scrotal masses,1
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RESULTS
The median age of the patients in this analysis was
55-years-old (range, 5- to 79-years-old). The median length
Local-regional Control
There were no local recurrences in this group of 15 patients.
Two patients suffered from regional metastases to the ipsilateral
pelvic nodes. In one case, the patient had exploration and gross
total resection with positive margins before RT. In the other case,
the patient had exploration with gross residual disease, followed
by planned management and was disease-free for 4 years. Unfortunately, he then had 4 regional recurrences, which were
managed surgically before any additional RT was given.
Survival
Only 2 patients died of disease: 1 who experienced
multiple regional recurrences and expired after his sixth attempted surgical resection and the second from a distant
metastasis to the lung, after suffering from delayed diagnosis
Complications
Few complications were found in our study despite the
sensitive nature of the proximal medial thigh being recognized
for high risks of combined radiotherapy and surgical complications. Complications were scored using the National Cancer
Institutes Common Terminology Criteria for Adverse Events,
version 4.0.3 We had 2 patients with chronic pelvic pain (grade
2), 1 patient with cellulitis requiring incision, drainage, and
antibiotics (grade 3), and 1 patient with bilateral lower-extremity
lymphedema (grade 2).
DISCUSSION
Our results demonstrate that spermatic cord sarcomas are
unlikely to metastasize as compared with soft-tissue sarcomas of
the extremities and other sites and that their growth and development tend to be more indolent. In addition, these tumors are
usually found earlier in the disease process compared with sarcomas of other sites due to their sensitive location, which may
positively affect their outcomes. Ultimately regional control and
survival are exceptional for this group of sarcomas. Recurrences
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Hazariwala et al
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question. Thus, if the rates of nodal failure are indeed low, then
the extent of the low pelvic radiation portals can be minimized
as well as the boundaries of the surgical resection, thereby
decreasing potential treatment toxicity. However, such a study
would require a large patient pool in order to adequately power
the endpoints and, given the rarity of these sarcomas, it is
unlikely that such a study will be conducted. Our study looked
at both patients treated preoperatively and postoperatively as
well as patients who did and did not undergo definitive surgery. Given the natural history of this disease, a longer period
of follow-up would be beneficial to determine long-term outcomes of local-regional control, cause-specific survival, and
integrity of the treated tissues.
As a retrospective case series, this study has several
limitations, despite the potential value of such a study design.
First, one must depend on the accuracy of the medical record
and its availability. The data in the chart are also subject to
patient recall bias. In addition, the study is prone to selection
bias, as the investigator is the one to choose the cases. Finally,
there are no control cases in this study. Further studies comparing patients treated solely with surgery versus those treated
with neoadjuvant radiation and surgery may be of value. Alternatively, additional studies examining various radiation
fields may help to determine if some toxicities can be limited
or if outcomes can be further improved.
CONCLUSIONS
Proper evaluation and diagnostic staging before initiating
planned treatment is critical, which can allow proactive management comparable with that of sarcomas of other primary
sites. Unplanned management, as we have seen, greatly complicates appropriate therapy and increases the risk of contamination around the tumor site and thus local-regional
recurrence. Compared with other sarcomas, most patients
survive their disease and die of unrelated causes, likely due to
early detection secondary to its location and readily apparent
symptoms. However, improper management can severely
compromise quality of life for these patients, and delayed
treatment carries a grave prognosis. To avoid such adverse
outcomes and morbidity, we strongly recommended planned,
preoperative radiotherapy followed by definitive resection
procedures as the optimal management strategy.
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