DIFFUSE UTERINE LEIOMYOMATOSIS: PRESERVING FERTILITY IN A YOUNG 23 YEAR OLD WOMAN.
Authors: Dr Hana Khan Ghori, under the guidance of Dr Madhura Phatak.
Manipal Hospitals- Mysore India.
Introduction
•Leiomyomas are common in gynaecological practice as a benign well circumscribed smooth muscle tumour. Diffuse Uterine Leiomyomatosis (DUL) on the other hand is a different entity.
•It is a rare, benign condition where there is symmetrical enlargement of the uterus due to multiple poorly defined confluent smooth muscle tumours replacing the myometrium.
•There have been less than 50 incidences of this condition reported in literature up till now.
Case Report
•In June 2021 a 23-year-old female presented with lower abdominal swelling. On examination, uterus was of 24 weeks size. The patient had no complaints of menorrhagia or dysmenorrhea.
•Imaging was done which showed multiple leiomyomata and an enlarged uterus.
•Options were discussed and she agreed on Abdominal Myomectomy. During the surgery it was found that she had multiple fibroids ranging from 0.5cm to 5 cm (as seen in image 1) as well as diffuse symmetrical enlargement of the uterus. Multiple leiomyomas were removed.
•The histopathological findings revealed benign tumours characterised by intersecting fascicles of monotonous spindle cells of smooth muscle with cigar-shaped nuclei, tapering ends, and eosinophilic cytoplasm (as seen in images 3a and 3b).
•She was then started on GnRH analogues for 6 months. During this the uterus reduced to regular size.
•Post discontinuation, in January 2023 she presented with abdominal swelling and uterus the size of 24 weeks for which she was referred to a higher centre for further management. MRI findings showed an enlarged uterus 21 x 16.5 x 6.8 cm with heterogeneous signal intensity and multiple hypointense lesions signifying recurrence of DUL (as seen in images 2a,2b,2c).
•She then underwent B/L Internal Iliac Artery Ligation and Laparoscopic Myomectomy with Hysteroplasty.
Discussion
•Aetiology of Diffuse Uterine Leiomyomatosis is not fully understood owing to the rare nature of the condition.
•Diagnosis is typically based on imaging studies like MRI and confirmed through histopathological examination.
•Clinical presentation may vary but common symptoms include abdominal distension, infertility, abnormal uterine bleeding, menorrhagia, dysmenorrhea, and pelvic pressure.
•Histopathological examination is crucial for confirming the diagnosis, showing spindle-shaped smooth muscle cells with interlacing fascicular arrangement, ill-defined cell borders, eosinophilic cytoplasm, cigar-shaped nuclei, small nucleoli, and low mitotic activity.
•Treatment options depend on fertility desires. Hysterectomy is a permanent solution, while conservative options include hormonal therapy and minimally invasive laparoscopic myomectomy. Uterine Artery Embolisation is also an option.
•Uterine leiomyomatosis can be associated with renal cell carcinoma in an Autosomal Dominant condition called HLRCC (Hereditary Leiomyomatosis Renal Cell Carcinoma).
•Screening for renal cell carcinoma is important in all cases of uterine leiomyomatosis.
Conclusion
Diagnosing and managing uterine leiomyomatosis are crucial to make informed decisions regarding patient care. This case underscores the challenging dilemma faced by a young patient desiring fertility preservation despite the absence of immediate pregnancy prospects. Both medical and surgical interventions were implemented to preserve fertility.
References
1. Prasad I, Sinha S, Sinha U, et al. (September 26, 2022) Diffuse Leiomyomatosis of the Uterus: A Diagnostic Enigma. Cureus 14(9): e29595. doi:10.7759/cureus.29595
2. Silva CA, Rosa F, Rito M, Cunha TM. Diffuse leiomyomatosis: A rare cause of a diffusely enlarged uterus. Radiol Case Rep. 2022 Mar 7;17(5):1536-1539. doi: 10.1016/j.radcr.2022.02.029. PMID: 35282327; PMCID: PMC8908024.
3. Layla, Hassan & P, Beoku-Betts & A, Oyawoye. (2020). Primigravida with diffuse uterine leiomyomatosis and IUGR necessitating a caesarean section and hysterectomy. Journal of Surgery and Surgical Research. 6. 084-086. 10.17352/2455-2968.000103.
