Document Type : Case Report Article
1 Departments of Women's Oncology, School of Medicine, Isfahan University of Medical Science, Isfahan, Iran
2 Departments of Pathology, School of Medicine, Isfahan University of Medical Science, Isfahan, Iran
Rhabdomyosarcoma (RMS) is a rare type of sarcoma. Botryoid type is the most common. The peak incidence is the second decade. Cervical RMS is rare in adults. We reported two cases, A, 31 years old woman G1L1 referred to a gynecologist oncologist with abnormal vaginal bleeding. And pathology reported RMS. She received 8courses of VAC regimen for chemotherapy .3 weeks after the last course, she underwent conization, and a remnant of sarcoma was seen. A hysterectomy was done for her. She has been disease free for 12 month. Case 2 was a 25 years old nulliparous woman presenting with abnormal vaginal bleeding, vaginal discharge, and cervical polypoid grape like lesion. Pathology reported RMS. Conization was done. Chemotherapy with VAC regimen was done for 8 courses. She has been disease free after 9 years. These two cases showed that RMS can occur in rare places and older ages.
Cervical sarcoma is a rare tumor, and also, RMS is a rare malignant skeletal muscle tumor that is rare (1). The embryonal subtype has been subdivided into classic, botryoid, and spindle cells (2). Cervical embryonal RMS are often a type of botryoid variant (3). The most typical presenting symptom is vaginal bleeding.
31 years old woman G1L1 presented with a complaint of abnormal vaginal bleeding. She was referred to a gynecologist oncologist because of a cervical lesion. The polypoid mass grape-like lesion was hyperemic and fragile 3 cm in diameter and the cervix's anterior lip in colposcopy examination. Biopsy was done, and Botryoid sarcoma was reported (Figure 1, 2). Microscopic examination revealed small round or spindle cells in a rather myxoid stroma. RMS differentiation as abundant eosinophilic cytoplasm and eccentric nuclei was seen in some cells. Tumor cell condensation beneath the squamous epithelium, forming the cambium layer of Nicholson, was evident.
Ct imaging of thorax and abdominopelvic was normal. She received eight courses of vincristine, actinomycin, cyclophosphamide (VAC) regimen. Vincristine (1.5 mg/m2, iv, d1), Dactinomycin (0.5 mg/kg, iv, d1-d5), cyclophosphamide (300 mg/m2, iv, d1-d5) every four weeks. The total length of treatment usually ranges from 6month to a year.
Three weeks after the end of chemotherapy, the growth appearance of the cervix was highly suspicious for tumor. (Figure 3) She was scheduled for conization surgery, endocervical curettage, and endometrial biopsy. Pathology reported minimal residue of cancer. So, the patient candidate for hysterectomy and underwent a hysterectomy. The margin of surgery was free of tumors.
Physical examination was done every 3 months, and she had no pathologic signs. In the first two years, we visit the patient every three months. The visit interval can be increased to six months, and the follow-up can be continued for five years. She has been disease free for 12 months.
Figure 1. Exophyitic lesion on colposcopic examination.
A 25 years old nulliparous woman complained of abnormal vaginal bleeding and vaginal discharge from four months earlier. Her endocervical and endometrial curettage was reported normal 2 months ago. In the vaginal examination, the cervical polypoid grape like mass lesion 36×63 mm was seen. Biopsy was done, and Botryoid sarcoma was reported (Figure 4, 5). As rapid regrowth of cervical tumor in three weeks happened, cervical conization surgery was done under anesthesia. No tumor residues were reported in pathology, and the chemotherapy, including the (VAC) regime, was started. Chemotherapy continued for 3cycle. She was amenorrheic for 12 month and FSH level was 100 IU/L (1.09-9.2 IU/L). But menstruation restarted regulated after that.
2 weeks after chemotherapy, ECC and EMC were done, and pathology was reported normal. In follow-up, every 3 months, she has no pathologic signs, and after 9 years, she has been disease free with no recurrence.
Figure 4. Medium power view (x 100): cambium layer
Figure 5. Medium power view (x 100): nodules of immature cartilage within the tumor.
We present one case of 31 years woman G1L1with cervical RMS diagnosed in a cervical polyp and the other case 25 years old nulliparous woman with cervical mass.
Visual inspection of the cervix in these patients was abnormal. The diagnosis was only confirmed after a biopsy and pathology exam. These two cases showed that RMS could occur in rare places and older ages. Adults are more likely to resist chemotherapy.
RMS are rare in adults; compared to children, it accounts for half of the sarcoma cases (1). In patients, cervical RMS presents as a cervical mass or polyp” (4).
Immunohistochemistry stains can aid in diagnosis. Myogenin was the most specific and sensitive marker for RMS (5).
The differential diagnosis for cervical polypoid lesions in women includes endometriosis, cervical or endometrial polyps, adenofribroma, adenosarcoma, and lymphoma (3).
Approach to management have varied over the years, but radical surgery, for primary management, has progressively been replaced by chemotherapy (VAC) and more conservative surgery (6).
Standard treatment in pediatric is chemotherapy and fertility preservation.
Chemotherapy with VAC and radiation therapy concurrently may start on week4 of treatment (7). A SEER study showed that the five years overall survival in adults with RMS was 27% compared to 61% in children (8).
Although adults with RMS had generally worse survival than children, Cervical botryoid RMS has better survival and prognosis than vaginal lesions in children. The survival rate of vaginal and cervical lesions has been reported to be 96% and 60% (9).
These cases showed that adult patients with RMS might not present the typical cervical lesion. Also, more data on the treatment and survival of adults with cervical RMS are needed.
In conclusion, these cases showed that adult patients with RMS might not present with the typical cervical lesion. Also, RMS can occur in rare places and at an older age, and fertility preservation is possible. More data on the treatment and survival of adults with cervical RMS are needed.
We express our gratitude to patients who consented to participate.
The authors state that they do not have a conflict of interest and do not oppose the publication of this study.
This paper has no sponsor.
The authors declared no conflict of interest.