|LETTER TO THE EDITOR
|Year : 2015 | Volume
| Issue : 1 | Page : 161
A rare case of recurrent intravenous leiomyomatosis: Role of GnRH analogues?
S Kaur1, HB Tongaonkar2, A Maheshwari3, S Menon4
1 Department of Gynecological Oncology, Dharamshila Cancer Hospital, Delhi, India
2 Urogynecology Oncology, PD Hinduja Hospital, Mumbai, India
3 Gynecologic Oncology, Tata Memorial Centre, Mumbai, India
4 Department of Pathology, Tata Memorial Centre, Mumbai, India
|Date of Web Publication||3-Feb-2016|
Department of Gynecological Oncology, Dharamshila Cancer Hospital, Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kaur S, Tongaonkar H B, Maheshwari A, Menon S. A rare case of recurrent intravenous leiomyomatosis: Role of GnRH analogues?. Indian J Cancer 2015;52:161
Intravenous leiomyomatosis (IVL) is a rarely encountered benign smooth muscle cell tumor arising either from a uterine leiomyoma or from the walls of a uterine vessel and extending into venous channels. A 32-year-old multiparous female presented in July 2008 with the complaint of breathlessness since three months. She had an abdominal hysterectomy (with ovarian preservation) and excision of retroperitoneal mass one and a half years ago. The histopathology was IVL. Computer tomography scans in 2008 revealed a 9.7 × 5.7 cm pelvic mass arising from the right side of pelvis with hypodense thrombus along the entire extent of inferior vena cava (IVC) [Figure 1]. A two stage surgery was done. In the first sitting after cardiopulmpnary bypass inferior venacava thrombus was removed. The second surgery which included the excision of pelvic mass was done a month later. The histopathology of the IVC thrombus and the pelvic mass was recurrent IVL [Figure 2]. Two years later in November 2010, follow-up revealed a 7 × 6.3 × 7.7 cm well-defined multiloculated mass in the pelvis with enlarged common iliac nodes bilaterally and focal thrombosis of the right common iliac veins. On PET-CT there were hypermetabolic iliac nodes, and non-flourodeoxyglucose avid pelvic mass and subcentimetre size pulmonary nodules. Biopsy of the pelvic mass suggested a recurrence. Pulmonary nodules were too small to be biopsied. The patient declined further surgery for the same, so she was started on Gonadotropin-releasing hormone analogues (GnRH)s (Inj. Leuprolide 11.25 mg, three monthly). After the first dose the patient had complete regression of pelvic mass, iliac nodes as well as the lung nodules and partial recanalization of common femoral veins. She was then continued with the same treatment for preventing recurrence and is disease free on two years follow-up.
|Figure 2: Spindle cells arranged in short fascicles with hypocellular areas of extensive hyalinisation|
Click here to view
IVL was first recognized at the turn of century by Birch-Hirschfeld and has been sporadically reported since then.
 Intravenous tumor extension is usually confined to pelvic veins but very rarely progress to inferior vena cava and even to the heart. Worley et al. retrospectively reviewed four patients with IVL with intracardiac extension. They reported that surgery is the mainstay of treatment. At a mean follow-up of 25.5 months all the four patients were (100%) free of recurrence. Biri et al. reported a case of IVL presenting as recurrent pelvic mass but there was no intracardiac extension. Our patient was unique with multifarious presentation (retroperitoneal mass, intracardiac extension, recurrent pelvic mass, venous plus nodal recurrence, and pulmonary metastasis) and recurring twice in-spite of complete surgical removal previously.
Surgery is the treatment of choice, and complete excision should be attempted due to its recurrent though benign nature. This case report suggests a role of GnRH agonists in treatment and as maintenance therapy, which needs further evaluation.
To conclude, IVL should be suspected in middle-aged women with history of uterine leiomyoma presenting with cardiac symptoms and right atrial mass. There are few unresolved issues regarding the management of disease. Is complete surgical resection enough? Is there a role of hormonal therapy for maintenance? Hence further research in the subject is provoking.
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Worley MJ Jr, Aelion A, Caputo TA, Kent KC, Salemi A, Krieger KH, et al
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[Figure 1], [Figure 2]
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