1. Dedifferentiated Paratesticular Myxoid Liposarcoma :
A Case Report And Review Of The Literature
Dorothy Sparks MD, Tisha Farrell DO, Saleem Umar MD, Abdul Ghani MD, FACS
Western Reserve Care System/NEOUCOM Program
Youngstown, Ohio
Introduction
We describe the presentation of a patient with a rare dedifferentiated myxoid liposarcoma with four separate tissues type identified histologically. Even more unusual, this sarcoma was incidentally found in the paratesticular space arising from the spermatic cord.
Sarcomas are most frequently described as being found in the extremities or in the retroperitoneal space. Less than 1% of all sarcomas are found in the paratesticular space and indeed, fewer than 100 cases have been reported of liposarcomas found within this location. We review the literature documenting this rare phenomenon as well as report on the presentation and clinical course of our patient.
Case Report
The patient is a 67 year old male who originally presented to his PCP with the complaint of painless, right sided scrotal enlargement for the past year. The patient had only sought treatment after the scrotum’s size became an encumbrance to his daily activities. He had no systemic symptoms.
Physical examination revealed a healthy, well nourished Caucasian male with uncircumcised genitalia and a disproportionably large right scrotum. The patient was scheduled for an elective right inguinal herniorrhaphy.
On approaching the scrotal sac a large soft tissue mass was found. The incision was extended inferiorly across the superior aspect of the scrotum. Dissection from the scrotal wall revealed a well circumscribed mass with a normal testicle adhered to the mass. A high ligation of the spermatic cord was achieved. There was no infilatration of the cord in the inguinal area. No inguinal or ilial lymphadenopathy was appreciated.
The patient made a satisfactory post-operative recovery. Clinic follow-up in the interim has revealed a well healed surgical site with no sign of reoccurance.
Micro & Macroscopic Findings:
Discussion
The word “sarcoma” comes from the Greek translation meaning “fleshy growth”. Sarcomas are defined as neoplasms arising from mesenchymal tissue. On average 5000 new cases of soft tissue sarcomas are identified yearly---with the liposarcoma subtype comprising only 1000 of these cases. Often occuring in males between the ages of years, liposarcomas are most often associated with a presentation in the retroperitoneal space or on the extremities. Liposarcomas comprise only 7% of all paratesticular sarcomas with fewer than 100 cases dedifferentiated cases described in the literature to date.
Because of their insidious onset, paratesticular liposarcomas are rarely diagnosed preoperatively. As in our case, most have been mistaken for inguinal hernias or hydroceles. Differential diagnoses should include inguinal hernias and hydroceles as well as: epidermoid cysts of the testis, testicular abscess, hematoma, and neoplasms.
Conclusion
Paratesticular liposarcomas have no associated risk factors. The dedifferentiated, myxoid, and pleomorphic types of liposarcoma carry a significantly worse prognosis than the most common type, well differentiated liposarcoma. Liposarcomas can originate from a variety of structures within the paratesticular space with the spermatic cord being the most common. Work-up should include a testicular ultrasound as well as CT or MRI to evaluate the extent of disease. Surgical resection is the treatment of choice with possible scrotectomy, and/or nodal dissection. Historically, these masses are radiosensitive but show poor response to chemotherapy. Regardless of treatment, paratesticular liposarcomas carry a high risk of reoccurrence and require vigilant long term follow-up.
Figure 1. Intraoperative gross specimen.
Figure 2. Intraoperative view of in situ mass.
Figure 3. Gross Photo of encapsulated, multinodular paratesticular mass with a yellow-tan firm and gelatinous cut surface measuring 22.0 x 18.0 x 9.0 cm.
Figure 4. Dedifferentiated Liposarcoma having the appearance of a Fibrosarcoma
Figure 5. Dedifferentiated Liposarcoma with Malignant Fibrous Histiocytoma like appearance
Figure 3. Dedifferentiated Liposarcoma with abrupt transition from low grade liposarcoma to MFH like area