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Case Report = Спостереження з практики = Наблюдение из практики
UDC 616-006.363.04:616-006.6-033.2:616.711.5
Metastatic leiomyosarcoma of the thoracic spine: case report
Cherkaoui Mandour, Adil Belhachmi, Jawad Laaguili, Brahim El Mostarchid
Leiomyosarcoma is a rare malignant mesenchymal tumor arising from smooth muscle cells. Metastatic spreading is possible, which even rarely metastasize to the bone being the latter localization a delayed manifestation of its natural progression. Spinal metastases represent the most common bone localizations with prevalence in the thoracic and lumbar spine. We present a 55-year-old female, operated there's 2 years for a hysterectomy of uterine leiomyosarcoma, admitted in our department with a flaccid paraplegia. Spine magnetic resonance imaging showed an extradural mass, iso-intense on T2, enhancing after gadolinium administration; placed at T5-T6 level with a severe cord compression. The patient underwent a decompressive laminectomy T5 and a total removal of the tumor. The histological examination revealed a metastatic leiomyosarcoma. The prognosis of patients with leiomyosarcoma is variable depending on the resectability and existence of metastasis. The essential treatment is surgery but an additional therapy, such as radiotherapy and/or chemotherapy, may be required to improve local control. Key words: leiomyosarcoma; metastasis; thoracic spine. Ukrainian Neurosurgical Journal. 2015;(4):76-8.
Department of Neurosurgery, Military Hospital Mohammed V, Rabat, Morocco
Received, November 1, 2015. Accepted, November 27, 2015.
Address for correspondence:
Cherkaoui Mandour, Department of Neurosurgery, Military Hospital Mohammed V, Rabat, PC: 10000 Morocco, e-mail: mandour1978@hotmail.com
Метастатична лейомюсаркома грудного вщдшу хребта (спостереження з практики)
Cherkaoui Mandour, Adil Belhachmi, Jawad Laaguili, Brahim El Mostarchid
Вщдтення нейрохiрургN, Вшськовий госглталь Мохаммеда V, Рабат, Марокко
Надiйшла до редакцп 01.11.15. Прийнята до публiкацií 27.11.15.
Адреса для листування:
Cherkaoui Mandour, Department of Neurosurgery, Military Hospital Mohammed V, Rabat, PC: 10000 Morocco, e-mail: mandour1978@hotmail.com
Лейомюсаркома e рщюсною злояюсною мезенх1мальною пухлиною гладеньких м'яз1в. Можливе метастазування. Метастази в юстки - вкрай рщккш, спостер1гают в тзньому перюд1 природного переб1гу лейомюсаркоми. Серед ккткових метастаз1в найчаст1ше виявляють метастази в хребет з переважною Ух локал1зац1ею в грудному та поперековому вщдтах.
Ми представляемо 55-р1чну жшку, яку госп1тал1зували з млявою параплепею через 2 роки тсля пстеректомп з приводу лейомюсаркоми матки. Магшторезонансна томограф1я хребта показала екстрадуральне вогнище з 1зо1нтенс1вним сигналом на Тп, котрий посилився тсля введення гадолЫю; розташоване на р1вн1 TV-TVI з важким здавленням спинного мозку. Патенту проведена декомпрессивная ламшектомш на р1внi TV i повне видалення пухлини. Пстолопчне дослщження виявило метастаз лейомюсаркоми.
Прогноз для пац1ент1в з лейомюсаркомою залежить вщ резектабельност1 пухлини i наявност1 метастаз1в. Основним методом лкування е х1рурпчний, але можливе застосування додатковоУ' терапп, такоУ' як променева та / або х1мютерап1я, з метою полтшення контролю над пухлиною.
Ключовi слова: лейомюсаркома; метастази; грудний вддл хребта.
Укр. нейрохiрург. журн. — 2015. — №4. — С.76-78.
Метастатическая лейомиосаркома грудного отдела позвоночника (наблюдение из практики)
Cherkaoui Mandour, Adil Belhachmi, Jawad Laaguili, Brahim El Mostarchid
Отделение нейрохирургии, Военный госпиталь Мохаммеда V, Рабат, Марокко
Поступила в редакцию 01.11.15. Принята к публикации 27.11.15.
Адрес для переписки:
Cherkaoui Mandour, Department of Neurosurgery, Military Hospital Mohammed V, Rabat, PC: 10000 Morocco, e-mail: mandour1978@hotmail.com
Лейомиосаркома является редкой злокачественной мезенхимальной опухолью гладких мышц. Возможно метастазирование. Метастазы в кости - крайне редки, наблюдаются в позднем периоде естественного течения лейомиосаркомы. Спинальные метастазы представляют собой наиболее встречающуюся локализацию костных метастазов с преобладанием в грудном и поясничном отделах позвоночника. Мы представляем 55-летнюю женщину, которая поступила в отделение с вялой параплегией через 2 года после гистерэктомии по поводу лейомиосаркомы матки. Магниторезонансная томография позвоночника показала экстрадуральный очаг с изоинтенсивным сигналом на Тп, усылившийся после введения гадолиния; расположенный на уровне Ту-Ти с тяжелым сдавлением спинного мозга. Пациентке проведена декомпрессивная ламинэктомия на уровне Т„ и полное удаление опухоли. Гистологическое исследование выявило метастаз лейомиосаркомы.
Прогноз для пациентов с лейомиосаркомой зависит от резектабельности опухоли и наличия метастазов. Основным методом лечение является хирургический, но возможно применение дополнительной терапии, такой как лучевая и/или химиотерапия, с целью улучшения контроля над опухолью. Ключевые слова: лейомиосаркома; метастазы; грудной отдел позвоночника.
Укр. нейрохiрург. журн. — 2015. — №4. — С.76-78.
© Cherkaoui Mandour, Adil Belhachmi, Jawad Laaguili, Brahim El Mostarchid, 2015
ISSN 1810-3154 (Print). yKpaÏHCbKHH HenpoxipyprwHHH xypHan, 2015, №4
77
Introduction: Leiomyosarcoma is a rare mesenchymal malignant tumor arising from smooth muscle cells [1]. The most common localization of this neo- plasm, which accounts from 7% of soft tissue sarcomas [2; 3]. Metastatic spreading is possible, the most commonly reported sites of metastasis from leiomyosarcoma are the lungs, liver, kidney, brain and skin. Spinal metastases of leiomyosarcoma have rarely been reported [4]. We report a case of leiomyosarcoma metastasizing to the thoracic spine.
Case report: A 55-year-old female, operated there's 2 years for a hysterectomy for uterine leiomyosarcoma and chemotherapy, admitted in our department with a 01 month history of gradually worsening back pain and weakness in the lower limbs with urinary leakage and constipation. Neurological examination revealed a flaccid paraplegia, sensitive level T5-T6 with signs of pyramidal irritation. Spine magnetic resonance imaging (figure A; B) showed an extradural mass, hypo-intense on T1-weighted and iso-intense on T2-weighted, enhancing after gadolinium administration; placed at T5-T6 level with a severe cord compression. The patient underwent a decompressive laminectomy T5 and a total removal of the tumor (figure C). The histological examination revealed a clear evidence of a metastatic leiomyosarcoma.
After surgery, the patient did not present any neurological improvement and she benefited from a functional reeducation with chemo-radiotherapy.
Discussion: Leiomyosarcoma is a rare malignant tumor; but it has a strong potential for metastasis to
distant sites due to propensity for hematogenous spread [5]. Osseous metastases are rare and spinal metastases represent the most common bone localizations [6; 7] with prevalence in the thoracic and lumbar spine [5].
Histological examination is a reliable and important method for the confirmation of the diagnosis. Leiomyosarcoma present a wide range of atypia extending from a well differentiated pattern to an extremely anaplastic one, the latter typical of the most aggressive sarcomas. Cell morphology is quite similar to the benign leiomyoma, consisting in bundles of spindle- shaped, smooth cells with oval nucleus and long, slender bipolar cytoplasmic processes. Nuclear atypia, high mitotic index and zonal necrosis confirm the malignant nature of the neoplasm [8; 9]. Usually, leiomyosarcoma cells are positive for smooth muscle actin, weakly positive for desmin and negative for S100 protein [10].
From a review of the literature, we found that primary leiomyosarcoma arising from the spine and paravertebral muscles are very rare entities with only 14 cases reported according to the best of our knowledge [5; 11; 12]. Similarly, metastatic Leiomyosarcoma with spinal localization represent very uncommon findings with just few cases described in literature as either first presentation or secondary recurrence [13; 14]. In our case, the diagnosis was easy, because the patient had a history of hysterectomy for uterine leiomyosarcoma.
On magnetic resonance imaging, it presents as a hypo-iso-intense homogeneous signal on T1-weighted whereas a hyper-intense signal is generally evident in T2-weighted [15]. Differential diagnosis includes neu-
Figure A: Sagittal section of spine magnetic resonance imaging, showed an extradural mass, enhancing after gadolinium administration; placed at T5-T6 level with a severe cord compression.
Figure B: Sagittal section of spine magnetic resonance imaging, showed an extradural mass, iso-intense on T2-weighted with a severe cord compression.
Figure C: per-operative image showing laminectomy and tumor.
This article contains some figures that are displayed in color online but in black and white in the print edition
rinomas, neurofibromas, meningiomas, lymphomas, fibrous tumors, ependymomas and other metastatic tumors [11].
The prognosis of patients with leiomyosarcoma is variable depending on the resectability and existence of metastasis. Survival times range from weeks to 13 years [15]. Radiotherapy and/or chemotherapy after surgery may be required to improve local control, despite its relative resistance to this therapy.
Conclusion: Metastatic leiomyosarcoma represents an exceptionally rare finding among the metastatic spinal lesions. Surgery (decompression; spinal fixation) is recommended to reduce pain symptomatology and prevent from worsening of myelopathy.
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