|Year : 2019 | Volume
| Issue : 1 | Page : 36-38
Primary paravertebral low-grade fibromyxoid sarcoma
Binod Kumar Singhania1, Jummo Jini1, Gobinda Pramanick2
1 Department of Neurosurgery, Apollo Gleneagles Hospitals, Kolkata, West Bengal, India
2 Department of Radiology, Apollo Gleneagles Hospitals, Kolkata, West Bengal, India
|Date of Web Publication||11-Mar-2019|
Binod Kumar Singhania
Flat No. 10C, Block-9, Silver Spring, 5 J.B.S. Halden Avenue, Kolkata - 700 105, West Bengal
Source of Support: None, Conflict of Interest: None
Low-grade fibromyxoid sarcoma is a distinctive variant of fibrosarcoma. Despite its benign histological appearance, it is a tumor with malignant potential. Usually, it is occur in the proximal lower extremities and trunk. However, it is very rare in paravertebral region. The diagnosis is based on the histology and specific immunohistochemistry results. The treatment of choice is total excision followed by radiotherapy to prevent the possibility of recurrence. We excised the tumor to near total and a wide fixation has been done. Frozen section biopsy was misleading and reported a schwannoma.
Keywords: Fibromyxoid sarcoma, fibrosarcoma, immunohistochemistry, low-grade, paravertebral region, schwannoma
|How to cite this article:|
Singhania BK, Jini J, Pramanick G. Primary paravertebral low-grade fibromyxoid sarcoma. Apollo Med 2019;16:36-8
| Introduction|| |
Low-grade fibromyxoid sarcoma (LGFMS) was first described by Evans in 1987. It is a rare sarcoma exhibiting bland histological features, but aggressive behavior by showing high rate of local recurrence (33%) and metastasis (58%). They are known to occur in the deep soft tissues of the proximal extremities, inguinal region, neck, and chest wall.,, There are only few reported cases of spinal, intraspinal, and paraspinal tumor.
| Case Report|| |
A 46-year-old female presented with complaints of tingling and numbness from the umbilicus downward with progressive weakness of both lower limbs for the past 6 weeks and became paraparetic for 1 week with bladder and bowel involvement. She was operated for D8 and D9 spinal schwannoma by debulking through S-shaped incision at the native hospital 2 years back. The then histopatholgical report showed schwannoma. Her recent magnetic resonance imaging (MRI) with contrast showed a lesion involving the right 9th rib, D8, D9 vertebral bodies with D10 right pedicle [Figure 1]. A large intraspinal component which was compressing dura shifted the cord on the left side severely and a paraspinal mass on the right side reaching up to great vessel [Figure 2]. Seeing a large extent of tumor, to minimize intraoperative bleeding, preoperative embolization was planned. Selective spinal angiography showed moderate vascularity [Figure 3]a and a common blood supply to tumor as well as cord [Figure 3]b. Hence, embolization was not done in view of increased risk of cord ischemia. A lateral extracavitary and costotransversectomy approach was decided to excise the tumor from the same old incision as vertical incision may cause flap necrosis. Skin flap was raised and tumor was bulging from previous partial laminectomy. D6, D7, left D10, D11, and D12 pedicular screws put and left-sided rod was fixed. A complete D8 to D10 laminectomy was done and right-sided 9th rib has been excised along with head and transverse process. Intraspinal as well as paraspinal tumor was excised along with intravertebral portion. Paraspinal tumor appeared to be encapsulated and was not very vascular [Figure 4]a. A neuromonitoring was used during dural decompression. Frozen section biopsy of the tumor showed schwannoma. Being a benign lesion, gross total excision was done and en bloc vertebrectomy was not planned as very extensive surgery, i.e., D8 to D10. The remained bones of D8 and D9 vertebral bodies were very hard and a trough was made for expendable cage by chisel and nibbler. An appropriate-sized expendable cage was interpositioned at D8-D9 and finally the second rod was connected [Figure 4]b. No significant blood loss was recorded and the patient did not receive any blood transfusion. Postoperative period was uneventful and she showed improvement in her lower limb power from 2/5 to 4+/5 within 3–4 days and became ambulant within 1 week without any assistance. Final histopathology showed LGFMS. She was referred to an oncologist and a planned radiotherapy has been decided.
|Figure 1: (a and b) Magnetic resonance imaging with contrast, sagittal cut showing D8 and D9 vertebral bodies involvement with intraspinal extension|
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|Figure 2: (a and b) Magnetic resonance imaging with contrast axial cut showing para and intraspinal tumor severely compressed cord on the left side|
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|Figure 3: (a) Spinal angiography showing moderately vascular tumor. (b) Spinal angiography showing common feeder to tumor and cord|
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|Figure 4: (a) Intraoperative paraspinal tumor delivered in one piece. (b) Tumor-free dura with small old fibrous tissue with fixation and cage in situ|
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| Discussion|| |
LGFMS was first described by Evans as a slow-growing, asymptomatic soft tissue tumor with apparently benign histopathological characteristics harboring a malignant potential. Several new entities of LGFMSs have been described,, such as (1) low-grade myxofibrosarcoma, (2) LGFMS, (3) hyalinizing spindle cell tumor with giant collagen rosettes, and (4) sclerosing epithelioid fibrosarcoma. Myxofibrosarcoma may progress to high-grade sarcoma, but the remaining three types are almost always low-grade fibrosarcoma.
Fibromyxoid sarcoma is a slow-growing, painless, deep soft tissue mass ranging in size from 1 to 18 cm and an average of 8–10 cm. It is commonly developing in deep soft tissues of the lower extremities, particularly in the thigh. The following region can be affected in decreasing order of frequency: the chest wall/axilla, shoulder region, inguinal region, buttock, and neck., Rare cases have been reported in unusual sites such as the retroperitoneum, small-bowel mesentery, mediastinum, and paravertebral region.,,
The imaging characteristics of LGFMS are a heterogeneous MRI appearance with low to slightly high signal intensity on T1, heterogeneously low to high signal intensity on T2, and heterogeneous postcontrast enhancement.
In our case, the tumor showed heterogeneous postcontrast enhancement with central hypointense core. It was a well-circumscribed mass in the right paravertebral region measuring approximately 5.0 cm × 5.7 cm × 4.1 cm and extending into the spinal canal and right foramen with erosion of D8 and D9 vertebrae involving the posterior element and encasement of right D9 nerve root.
Grossly the tumor was grayish tan in color [Figure 5]. A cut section showed focal areas of glistening grayish-white substance with scattered partially seromucinous fluid. LGFMS diagnosis was made based on the fibroblastic characteristic shown by the tumor cells. They were spindle shaped with fusiform nuclei and faint eosinophilic cytoplasm lacking perinuclear vesicles. By means of immunochemistry, the cells did not show any lineage specificity (negative for B catenin, CD 99, EMA, S-100 protein, smooth muscle actin, and CK). CD34 was positive with Mib1 (ki67) expressed in approximately 50% at most active area, indicating that the tumor has a benign characteristic.
The differential diagnosis of LGFMS includes benign and malignant soft tissue lesions characterized by a fibrous and myxoid stroma.
| Conclusion|| |
Paraspinal tumor should be excised in totality whenever feasible to give the patient the best possible treatment and prevent future recurrence. Postoperative radiotherapy may be considered to increase the chances of a progression free survival.
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.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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