Although leiomyomas are common this case is being reported due to its uniqueness as it posed challenges at all levels of pathological rarity and management. Symplastic leiomyoma is an unusual benign variant of leiomyoma with less likelihood of malignant transformation. In our case report we present a 28-year-old nulligravida who presented to our OPD with Fibroid uterus detected on scan and myomectomy specimen done revealed a rare pathological diagnosis of symplastic leiomyoma and her followup

N., Usha

Priyadarshini, Sarah Grace

Shenoy, Kavitha V.

English

International Journal of Reproduction, Contraception, Obstetrics and Gynecology

                                                                                                                             November 2023 · Volume 12 · Issue 11    Page 3399 International Journal of Reproduction, Contraception, Obstetrics and Gynecology Shenoy KV et al. Int J Reprod Contracept Obstet Gynecol. 2023 Nov;12(11):3399-3402 www.ijrcog.org pISSN 2320-1770 | eISSN 2320-1789 Case Report Symplastic leiomyoma: a rare clinicopatholgical diagnosis Kavitha V. Shenoy1*, Usha N.1, Sarah Grace Priyadarshini2            INTRODUCTION Although leiomyomas are common this case is being reported due to its uniqueness as it posed challenges at all levels of pathological rarity and management. Symplastic leiomyoma is an unusual benign variant of leiomyoma with less likelihood of malignant transformation. Patient counselling is important to alleviate the anxiety associated with such histologic reports. Atypical and bizarre leiomyoma synonymous with symplastic leiomyoma are smooth muscle tumors that contain cells with moderate to severe cytological atypia, with no cell necrosis and mitotic index fewer than 10/10 HPF. It was also diagnosed in different sites other than uterus such as the vagina, nasal cavity, and scrotum. Malignant transformation accounts for 0.2% of all cases of leiomyomas.  CASE REPORT Mrs M, 28 years lady, married for 6 months, presented to OPD for opinion regarding fibroid uterus diagnosed on ultrasound on routine checkup. She had no history suggestive of abnormal uterine bleeding or abdominal distension. No history of weight loss, pain abdomen or dysmenorrhea, bowel or bladder complaints. On enquiring her mother had undergone hysterectomy in view of abnormal uterine bleeding-leiomyoma. Her sibling also has large leiomyoma. On examination-moderately built and nourished. No pallor and pedal edema. Vitals stable. Breast, thyroid and spine normal. Per abdomen examination-An irregular, bosselated, abdominopelvic mass corresponding to 30 weeks gravid uterus size, extending transversly from right lumbar region to left lumbar region occupying all quadrants of abdomen, firm in consistency, non tender, lower border not made out.  Per speculum examination revealed a healthy cervix and vagina. Per vaginal examination showed firm posteriorly deflected cervix with left forniceal fullness: mass felt in the pouch of douglas, non-tender, uterus corresponding to 30 weeks size. She was further evaluated. Blood investigations were normal. MRI pelvis done for fibroid mapping-revealed multiple fibroids-Left fundus-FIGO 5 transmural-isoechoic 8×8.5×7.8 cm, midline anterior fundus-FIGO 4-intramural isoechoic 3.4×4×4.2 cm, right lateral corpus-FIGO 5 transmural-hyperintense 11.3×10.5×10.4 cm, left lateral corpus FIGO 5 transmural-hyperintense DOI: https://dx.doi.org/10.18203/2320-1770.ijrcog20233318 1Department of Obstetrics and Gynecology, 2Department of Pathology, Vijaya Medical and Educational Trust, Chennai, Tamil Nadu, India  Received: 25 September 2023 Accepted: 19 October 2023  *Correspondence: Dr. Kavitha V. Shenoy, E-mail: kavithavshenoy@gmail.com Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Although leiomyomas are common this case is being reported due to its uniqueness as it posed challenges at all levels of pathological rarity and management. Symplastic leiomyoma is an unusual benign variant of leiomyoma with less likelihood of malignant transformation. In our case report we present a 28-year-old nulligravida who presented to our OPD with Fibroid uterus detected on scan and myomectomy specimen done revealed a rare pathological diagnosis of symplastic leiomyoma and her followup.  Keywords: Symplastic, Leiomyoma bizarre, Atypical leiomyoma  Shenoy KV et al. Int J Reprod Contracept Obstet Gynecol. 2023 Nov;12(11):3399-3402 International Journal of Reproduction, Contraception, Obstetrics and Gynecology                                 Volume 12 · Issue 11    Page 3400 4.8×4.4×5.2 cm and right lateral cervix-FIGO 8-posterolateral right cervix 7.5×6×5.8 cm-hyperintense.             Figure 1: MRI fibroid mapping showing multiple fibroid uterus. She underwent laparotomy by midline subumbilical vertical incision- -myomectomy done through tunnelling incision technique after bilateral ureteric catheterisation under combined spinal epidural anaesthesia. Intraoperatively Uterus was irregularly enlarged to 28 weeks gravid uterus size with multiple large fibroids-Fundal subserous fibroid FIGO V-8×8 cm, right lateral wall subserous fibroid FIGO V 10×10 cm, two intramural fibroids of 4×4 cm FIGO IV from anterior wall, along with multiple seedling fibroids. HPE revealed symplastic leiomyoma-Large hyperchromatic nuclei with chromatin clumps. Giant cells with multiple large nuclei and stroma with myxoid changes. There was no nuclear atypia/necrosis. Tumor was regular with low mitotic activity.  Figure 2: Intraoperative figure showing uterus with large leiomyoma.        Figure 3: Large fundal fibroid.  Figure 4: Enucleation of fundal fibroid during myomectomy.  Figure 5: Retrieved myomectomy specimens-five leiomyomas. Shenoy KV et al. Int J Reprod Contracept Obstet Gynecol. 2023 Nov;12(11):3399-3402 International Journal of Reproduction, Contraception, Obstetrics and Gynecology                                 Volume 12 · Issue 11    Page 3401  Figure 6: Spindle shaped neoplastic cells in fascicles (100x H and E), bizarre nuclear forms with giant cells. Follow-up Follow-up scan post 6 months post-surgery showed uterus AV 11×6×6 cm with small seedling fibroids and Polycystic ovaries. HSG was done which showed normal cavity with free spill on both tubes. She is currently on management for subfertility.  Figure 7: TVS scan showing uterus with endometrial cavity post-surgery.  Figure 8: Hysterosalpingogram post surgery showing normal uterine cavity with free spill on both tubes. DISCUSSION Numerous histologic variants of uterine leiomyomas are described. One such variant is the symplastic leiomyomaa-the term reserved for uterine leiomyomas with giant cells, nuclear atypia and, minimal mitotic activity (0-4/10 hpfs). The presence of atypia is well recognized. In 1966, Taylor and Norris first introduced the term ‘atypical’ uterine leiomyomas to denote the presence of cytologic atypia in uterine leiomyomas. Subsequently terminologies used for histologically similar lesions with cytologic atypia included ‘bizarre leiomyoma’ by Christopherson et al and, ‘symplastic leiomyoma’ by burns, suprarenal symplastic leiomyoma of the inferior vena cava, myxoid tumor of the uterus and right atrial myxomas benign metastatizing leiomyoma have been reported.1-5 The case of symplastic leiomyoma had previously been reported as a single case. The criteria used in order to consider a smooth muscle tumour as a leiomyosarcoma were: a mitotic rate of two or more mitoses per 10 high power field/presence of necrosis. CD 74 and p53 are two of the most studied markers. The following criteria were evaluated:6 Degree of cytologic atypia (none to mild or moderate to marked), presence or absence of coagulative necrosis and mitotic index (MI) (Table 1). Table 1: Criteria for evaluating various categories of smooth muscle neoplasm of the uterine corpus.6 Leiomyoma  MI < 20MF/10 HPF Leiomyoma with increased MI  No coagulative necrosis  No atypia or no more than mild cytological atypia Leiomyoma with increased MI, but experience limited MI ≥20MF/10 HPF  No coagulative necrosis  No atypia or no more than mild cytological atypia Atypical leiomyoma MI <10/10 HPF No coagulative necrosis  Diffuse moderate to severe cytological atypia Leiomyosarcoma Any MI  Any degree of cytological atypia with coagulative necrosis Epithelioid leiomyoma MI <5MF/10HPF No coagulative necrosis No atypia/more than mild atypia Epithelioid leiomyosarcoma MI ≥5 MF/10 HPF No coagulative necrosis None /any degree of cytological atypia The presence of hematopoietic or heterologous elements within an otherwise bland uterine leiomyoma or endometrial stromal tumor may give rise to diagnostic difficulties. The regularity of the tumor margins, low mitotic activity, and absence of nuclear atypia or necrosis should be made for the exclusion of malignancy. In the Shenoy KV et al. Int J Reprod Contracept Obstet Gynecol. 2023 Nov;12(11):3399-3402 International Journal of Reproduction, Contraception, Obstetrics and Gynecology                                 Volume 12 · Issue 11    Page 3402 presence of massive lymphocytic infiltration of a leiomyoma, the clonality of the infiltrate may aid in differentiating it from malignant lymphoma. The pathogenesis and clinical significance of these rare neoplasms remain to be clarified. CONCLUSION Symplastic leiomyoma is an unusual variant of leiomyoma. Malignant transformation accounts for 0.2% of all cases of leiomyoma. The regularity of the tumor margins, low mitotic activity and absence of nuclear atypia or necrosis should be made for the exclusion of malignancy. Funding: No funding sources Conflict of interest: None declared Ethical approval: Not required REFERENCES 1. Demirkan A, Ulas S, Atil K, Cakmak A, Demirer S, Alaçayir I et al. Suprarenal symplastic leiomyoma of the inferior venacava. Ann Vas Surg. 2009;23(6):786.e11-3.  2. Barlow JF, Abu-Gazeleh S, Tam GE, Wirtz PS, Ofstein LC, O'Brien CP et al. Myxoid tumor of the uterus and right atrial myxomas. S D J Med 1983;36(7):9-13. 3. Patton KT, Cheng L, Papavero V, Matthew GB, Anjana VY, Brian PA et al. Benign metastatizing leiomyoma. Mod Pathol. 2006;19(1):130-40. 4. Mamalingam M, Goldberg LJ. Atypical pilar leiomyoma: cutaneous counterpart of uterine symplastic leiomyoma? Am J Dermatopathol 2001;23:299-303. 5. Scully RE, Bonfiglio TA, Kurman RJ. Histologic Typing of Female Genital Tract Tumors. World Health Organisation International Histologic Classification of Tumors. Second edition. Berlin: Springer-Verlag. 1994. 6. Bell SW, Kempson RL, Hendrickson MR. Problematic uterine smooth muscle neoplasms: a clinicopathologic study of 213 cases. Am J Surg Pathol. 1994;18(6):535-58.       Cite this article as: Shenoy KV, Usha N., Priyadarshini SG. Symplastic leiomyoma: a rare clinicopatholgical diagnosis. Int J Reprod Contracept Obstet Gynecol 2023;12:3399-402. 

Symplastic leiomyoma: a rare clinicopatholgical diagnosis

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Symplastic leiomyoma: a rare clinicopatholgical diagnosis

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International Journal of Reproduction, Contraception, Obstetrics and Gynecology