|Year : 2017 | Volume
| Issue : 2 | Page : 252-254
Leiomyosarcoma with rhabdoid differentiation arising from leiomyoma: A rare entity
Pooja Chauhan, Kavita Mardi, Ganga Rawat, Nidhi Raina
Department of Pathology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
|Date of Web Publication||15-Dec-2017|
H. No. 51 Housing Board Colony, Sanyar, PO. Talyahar, Mandi - 175 001, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
Leiomyosarcoma (LMS) arising in a leiomyoma (LM) is a rare event. Still rare is rhabdomyoblastic differentiation in leiomyosacoma. We present such a case of LMS uterus with rhabdomyoblastic differentiation arising in an LM in a 60-year-old menopausal woman who presented with lower abdominal pain. An ultrasound scan was suggestive of uterine fibroid. Total abdominal hysterectomy was performed for presumed LM. Grossly, fibroid had variegated appearance along with focal areas having whorling pattern and histopathology showed leiomyosarcomatous change in a benign LM with rhabdomyoblastic differentiation. This is a rare complication of LM and precise diagnosis of LMS is essential since it has an aggressive behavior.
Keywords: Leiomyoma, leiomyosarcoma, rhabdomyoblastic differentiation, uterus
|How to cite this article:|
Chauhan P, Mardi K, Rawat G, Raina N. Leiomyosarcoma with rhabdoid differentiation arising from leiomyoma: A rare entity. Arch Med Health Sci 2017;5:252-4
|How to cite this URL:|
Chauhan P, Mardi K, Rawat G, Raina N. Leiomyosarcoma with rhabdoid differentiation arising from leiomyoma: A rare entity. Arch Med Health Sci [serial online] 2017 [cited 2018 May 25];5:252-4. Available from: http://www.amhsjournal.org/text.asp?2017/5/2/252/220834
| Introduction|| |
Uterine leiomyosarcoma (LMS) is the most common uterine sarcoma comprising of approximately 1% of all uterine malignancies. It is generally believed that uterine LMSs arise de novo as isolated lesions, rather than from any precursor lesions and overwhelming data strongly suggest it. However, rare cases supporting the hypothesis that LMSs may arise from preexisting leiomyomas (LMs) have been reported. We report such case of malignant transformation in LM along with rhabdomyoblatic differentiation in an elderly postmenopausal female. Despite the rarity of the event, aggressive nature of the tumor as compared to all the other uterine malignancies increased the tendency for local recurrence and the distant metastasis, all make it an important entity.
| Case Report|| |
A 60-year-old menopausal woman reported to gynaecology OPD with the complaints of the firm to hard nontender, gradually increasing abdominal mass and dull aching lower abdominal pain for the past 1 year. Over the past 3 months, there was a rapid increase in abdominal mass. There was no history of postmenopausal bleeding or vaginal discharge. On physical examination, she was generally well and vital signs were normal. Abdominal examination revealed abdominal distension. On pelvic examination, uterus was enlarged to 16–18 weeks size and the clinical impression was of uterine fibroid. The clinical finding was supported by ultrasound scan which revealed large uterine fibroid measuring 11 cm × 10 cm.
Total abdominal hysterectomy with bilateral salpingo-oophorectomy was done and sent to us for histopathological examination. There was a large intramural fibroid measuring 10 cm in diameter cut section of which was gray-white to gray-brown with focal yellowish/myxoid/hemorrhagic and necrotic areas [Figure 1]. Microscopic examination of the intramural fibroid showed typical areas of LM with extensive areas of hyalinization. However, there was an abrupt transition of these areas into areas comprising of sweeping and interlacing fascicles of spindle cells in a myxoid background. These spindled cells had elongated, hyperchromatic, and pleomorphic nucleus with blunt ends. Occasional intracytoplasmic vacuoles indented the nuclei. Extensive areas of necrosis, hemorrhage and atypical mitotic Figures >10/10 HPF were also seen. Scattered rhabdomyoblasts having concentrically arranged eosinophilic fibrillary material around the nucleus were also present [Figure 2]. The presence of transitional morphologic change from a LM to LMS was suggestive that LM might be the precursor of malignancy.
| Discussion|| |
LMSs are rare neoplasm with an annual incidence estimated to be at 0.64% of cases per 100,000 women in a study conducted by Harlow et al. The incidence of LMS in women with a preoperative diagnosis of fibroid uterus is between 0.13% and 0.29% as recorded by Leibsohn et al. Still rarer is LMS arising in an LM as was encountered in our case.
Mittal et al. examined 18 cases of uterine LMS associated with LM like areas at histological, immunohistochemical, and DNA level. They evaluated if benign looking LM-like areas were related to leiosarcomatous areas. In six cases, the LM-like and uterine LMS areas from each case were examined using high-density oligonucleotide array-comparative genomic hybridization to determine genetic aberrations in the two areas. Nearly, all genetic aberrations found in LM-like areas were also found in the corresponding uterine LMS areas though LMS had additional genetic aberrations.
Indraccolo et al. monitored a patient with three LM over the years. Two of these LM were transformed into LMS after menopause; this study supported the hypothesis of malignant transformation in a LM.
A study by Yanai et al. revealed that malignant transformation can occur even in relatively small LM and that LMS with an LM component has a favorable prognosis. They also found immunohistochemical examination of p53, p16, and Ki-67 useful for identification of malignant focus. As the frequency of LMSs is only 0.1%–0.3% of the frequency of LMs, only rare LMs progress to LMS.
Recent microassay data, however, identified a rare subset of myomas with deletions of chromosome-1 that have transcriptional profiles that cluster with those of LMSs  suggesting that some rare LMS may arise from a specific subset of myomas.
Transcriptional profiling has linked LM to malignancy through two genomic regions on chromosome 1. Mutation of fumarate hydratase at 1q43 and loss of short arm of chromosome 1 in cellular leiomyoma have been accounted for this malignant transformation.
Primary therapy for early-stage LMS in postmenopausal women is total abdominal hysterectomy and bilateral salpingo-oophorectomy. Therapy in premenopausal women is more controversial.
LMS is a highly malignant neoplasm with poor survival rates. For prognosis and predictive factors show that LMS is a highly malignant neoplasm and the overall 5-year survival rates range from 15% to 25%. In stage I and II tumors, it is 40%–70% according to the WHO. All malignant neoplasms with rhabdoid morphology are recognized as highly aggressive and show a tendency for poor prognosis.,
| Conclusion|| |
This is an uncommon documentation of a case of LMS arising on a background of LM and exhibiting rhabdoid differentiation. The value of identification of these components relates to radical treatment due to their aggressive clinical course. Value of optimal tissue sampling in such cases cannot be overemphasized.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]