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LETTER TO EDITOR
Year : 2016  |  Volume : 64  |  Issue : 5  |  Page : 1071-1074

Lumbar paraspinal primary high-grade leiomyosarcoma mimicking an extraforaminal schwannoma


1 Department of Neurological Sciences, Christian Medical College Hospital, Vellore, Tamil Nadu, India
2 Department of Pathology, Christian Medical College Hospital, Vellore, Tamil Nadu, India

Date of Web Publication12-Sep-2016

Correspondence Address:
Ranjith K Moorthy
Department of Neurological Sciences, Christian Medical College Hospital, Vellore, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.190226

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How to cite this article:
Gupta A, Moorthy RK, Prabhu AJ, Rajshekhar V. Lumbar paraspinal primary high-grade leiomyosarcoma mimicking an extraforaminal schwannoma. Neurol India 2016;64:1071-4

How to cite this URL:
Gupta A, Moorthy RK, Prabhu AJ, Rajshekhar V. Lumbar paraspinal primary high-grade leiomyosarcoma mimicking an extraforaminal schwannoma. Neurol India [serial online] 2016 [cited 2019 Nov 21];64:1071-4. Available from: http://www.neurologyindia.com/text.asp?2016/64/5/1071/190226




Sir,

Leiomyosarcomas (LMSs) are variably aggressive tumors of smooth muscle cell origin, with a high propensity to metastasize to various locations. They fall under the broad category of soft tissue sarcomas that constitute 0.7% of all malignancies. They have been reported to constitute 5–16% of all soft tissue sarcomas.[1],[2],[3] They commonly arise in the uterine wall and retroperitoneal soft tissue, and rarely arise from sites such as the somatic soft tissue (extremity and trunk), bone, and vascular tissue. We report a rare case of a lumbar paraspinal high-grade LMS that presented clinically and radiologically as a nerve sheath tumor. There are four cases of primary paraspinal LMSs that have been reported in the literature with none being lumbar in location.[4],[5],[6],[7]

A 34-year-old woman presented with low backache radiating along the posterior aspect of her right lower limb for 4 years that had worsened over the past 4 months. On examination, she had wasting of the right calf muscles and weakness of right ankle plantar flexion. The straight leg raising test was negative. Magnetic resonance imaging (MRI) of the lumbosacral spine revealed a right L4–L5 paraspinal mass extending proximally into the L4–L5 intervertebral foramen. The mass was isointense on T1-weighted images and hyperintense on T2-weighted images, with homogeneous enhancement on gadolinium injection [Figure 1]. There was no significant invasion into the adjacent structures or blood vessels. A diagnosis of extraforaminal schwannoma/neurofibroma was considered. She underwent a right L4–L5 paraspinal exploration and total excision of the tumor. Intraoperatively, the tumor was grayish, relatively avascular, firm, and nonsuckable, and it was located between the L4 and L5 transverse processes and adjacent to the L4 body anteriorly. The L4 nerve roots were dorsal to the tumor in the intervertebral foramen, and a few nerve roots appeared to be entering the tumor. It had a fairly good plane of cleavage from the surrounding muscles and paraspinal structures. Postoperatively, there was improvement in her back and radicular pain.
Figure 1: (a) T1-weighted axial magnetic resonance imaging section with gadolinium injection, showing homogenous contrast enhancement of an isointense tumor in the right L4–L5 paraspinal region. (b) T2-weighted axial magnetic resonance imaging section showing the hyperintense tumor (white arrow). (c) T1-weighted plain coronal magnetic resonance imaging section showing the iso-intense tumor (white arrow)

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The histopathology revealed the tumor to be composed of interlacing fascicles of spindle shaped cells with oval to elongated, moderate to marked pleomorphic nuclei, moderate amounts of eosinophilic cytoplasm, and indistinct cell boundaries. Few mitotic figures were seen. Foci of stromal myxoid degeneration were also seen. The tumor cells were immunopositive for smooth muscle actin and h-caldesmon (smooth muscle markers), and immunonegative for desmin (skeletal muscle marker) and S-100 (neurogenic marker). The MIB-1 (Ki-67) labeling index of the tumor was 50%. A pathological diagnosis of malignant spindle cell neoplasm, consistent with a high-grade LMS was made [Figure 2].
Figure 2: (a) Photomicrograph showing the pleomorphic spindle cells in fascicles (H and E, ×200). (b) Immunopositivity for h-caldesmon (smooth muscle marker, ×400). (c) MIB-1 proliferation index of 50%. (d) Spindle cells diffusely positive for smooth muscle actin (×100)

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She subsequently received 59 Gy of external beam radiotherapy and one cycle of ifosfamide and doxorubicin chemotherapy at another center. At a follow-up of 6 months, she was found to be cachexic and had significant pallor. She was detected to be having metastatic lesions in the left scapula with a subcutaneous nodule, the histopathology of which confirmed the lesion to be metastatic LMS. A computed tomographic scan of the abdomen and pelvis revealed systemic spread of the disease with involvement of the liver and lumbar vertebral bodies [Figure 3]. She was started on palliative chemotherapy with a single agent doxorubicin and was subsequently lost to follow-up.
Figure 3: (a and b) Multiple hepatic parenchymal lesions, suspicious of metastasis, detected during a 6-month follow-up (white arrows)

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The incidence of LMSs has been reported to be in the range of 5–16% of all soft tissue sarcomas.[1],[2],[3] The mean age of presentation of these tumors is around the sixth decade of life. There have been conflicting reports in the literature on the sex predilection of these tumors. In a review article on LMSs, the female:male ratio was 2:1, raising the speculation that estrogen could cause smooth muscle cell proliferation, and hence, a higher incidence of LMSs was found in female patients.[8] Our patient was a 34-year-old female. Her age was three decades younger than the mean age reported in the literature.

LMSs are evenly distributed over various locations in the body. Retroperitoneal LMSs are the most common lesions and are associated with a grave prognosis considering their metastatic potential. Somatic (or external) soft tissue LMSs are generally considered to be of intermediate metastatic potential and are sub-classified as cutaneous, subcutaneous, or deep soft tissue tumors, being present over the thighs, arms, or trunk.[3] Our patient had a paraspinal LMS, which is considered a deep soft tissue LMS. The 5-year survival rate of somatic soft tissue LMSs has been reported to be 61% compared to the 21% survival rate of retroperitoneal LMS.[2],[9] Previously published case series have documented <5 cases of lumbar soft tissue LMSs that were moderately differentiated, and there were no details available for these cases.[2] There have been three reported cases of cervical paraspinal LMSs and one of a thoracic paraspinal LMS [Table 1].[4],[5],[6],[7] However, to the best of our knowledge, this is the first report of a lumbar paraspinal LMS in literature.
Table 1: Overview of previously reported cases on primary paraspinal leiomyosarcomas

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LMSs arise from smooth muscle cells in the vicinity of blood vessels (veins and capillaries). It is probably for this reason that they have a high propensity to metastasize, considering their easy access to the vascular system. Histologically, these tumors consist of intersecting bundles of spindle-shaped cells, with typically elongated nuclei having blunt ends. The cytoplasm is eosinophilic and fibrillar, with occasional vacuoles seen within. The degree of nuclear pleomorphism varies from case to case. Immunopositivity to actin or desmin also aids in the diagnosis of LMSs.[2],[10]

It has been postulated that regulation of the phosphatidylinositol 3-kinase (PI3K), Akt (protein kinase B), and the mammalian target of rapamycin (mTOR) - the PI3K/Akt/mTOR signaling pathway - could play a critical role in smooth muscle transformation and LMS genesis.[11] Akt pathway upregulation causes overexpression of suppressor of cytokine signaling 3, which secondarily causes inhibition of signal transducer and activator of transcription 3 (STAT3). Immunohistochemical studies have shown that the presence of STAT3 phosphorylation could lead to a better prognosis among the soft tissue LMSs, and could be an important predictive marker in future.[12]

The magnetic resonance imaging (MRI) in our patient showed a right L4–L5 paraspinal mass, extending into the neural foramen. The common differential diagnoses of this tumor would be schwannoma, neurofibroma, meningioma, solitary fibrous tumor, metastasis, or a primary osseous neoplasm. The MRI characteristics of LMSs are very similar to those of a neurogenic tumor, in that they are hypointense on T1-weighted imaging, hyperintense on T2-weighted imaging and enhance homogenously with gadolinium injection. Hence, these tumors, particularly in the spinal and paraspinal regions, can easily be mistaken for nerve sheath tumors.[5],[7]

Currently, there is no consensus in the literature on the treatment protocol of these tumors, and there have been no randomized trials in this regard. However, it is generally accepted that a multimodality treatment comprising surgery and adjuvant radiotherapy and chemotherapy would provide the highest chances of survival. The prognostic factors in the management of LMSs are the histological grade of tumor, depth of tumor invasion, and age at presentation, with an age more than 60 years having a poorer prognosis.[13],[14],[15] Konomoto et al.,[16] have shown that abnormality of the p53 gene and a large tumor size (more than 6 cm) may also be a prognostic factor in soft tissue sarcomas. Wide surgical excision with adequate margins is critical in the initial management of the tumor, and this could be achieved in our patient. Postoperative external beam radiotherapy or low dose brachytherapy have shown similar results in achieving a local disease control.[17] Le et al.,[15] have shown that radiotherapy in doses ≤65 gray is inadequate in cases having a widespread disease. Despite appropriate postoperative radiotherapy and chemotherapy, these tumors show a tendency to recur with a median progression-free survival of 1.5 years [2] and an overall survival of 7.1 years.[3]

To conclude, the differential diagnosis of LMS should be borne in mind while dealing with paraspinal tumors, as this is an aggressive tumor with a propensity to metastasize. Although no consensus exists in the literature regarding their management, surgery followed by adjuvant chemotherapy and radiotherapy is the preferred treatment. However, despite aggressive treatment, they tend to recur either locally or at distant sites.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest

 
  References Top

1.
Gustafson P, Willén H, Baldetorp B, Fernö M, Akerman M, Rydholm A. Soft tissue leiomyosarcoma. A population-based epidemiologic and prognostic study of 48 patients, including cellular DNA content. Cancer 1992;70:114-9.  Back to cited text no. 1
    
2.
Hashimoto H, Daimaru Y, Tsuneyoshi M, Enjoji M. Leiomyosarcoma of the external soft tissues. A clinicopathologic, immunohistochemical, and electron microscopic study. Cancer 1986;57:2077-88.  Back to cited text no. 2
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3.
Mankin HJ, Casas-Ganem J, Kim JI, Gebhardt MC, Hornicek FJ, Zeegen EN. Leiomyosarcoma of somatic soft tissues. Clin Orthop Relat Res 2004;421:225-31.  Back to cited text no. 3
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4.
Aksoy DY, Altundag MK, Durusu M, Abali H, Onder S, Turker A, et al. Thoracic paravertebral leiomyosarcoma: Rare but it does occur. Spine (Phila Pa 1976) 2002;27:E301-3.  Back to cited text no. 4
    
5.
Lehman NL, Jacobs CD, Holsten PA, Jaikumar S, Lehman TD, Gibbs IC, et al. Primary paraspinal leiomyosarcoma invading the cervical spinal canal successfully treated with surgery, radiotherapy, and chemotherapy. Case report. J Neurosurg Spine 2007;6:441-6.  Back to cited text no. 5
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6.
Marshman LA, Pollock JR, King A, Chawda SJ. Primary extradural epithelioid leiomyosarcoma of the cervical spine: Case report and literature review. Neurosurgery 2005;57:E372.  Back to cited text no. 6
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7.
SenGupta SK, Nag S. Cervical paravertebral leiomyosarcoma mimicking a nerve sheath tumor. Hum Pathol 1992;23:708-10.  Back to cited text no. 7
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8.
Weaver MJ, Abraham JA. Leiomyosarcoma of the bone and soft tissue: A review. ESUN 2007;V4N2. Available from: http://www.sarcomahelp.org/leiomyosarcoma.html. [Last accessed on 2016 Aug 12].  Back to cited text no. 8
    
9.
Hashimoto H, Tsuneyoshi M, Enjoji M. Malignant smooth muscle tumors of the retroperitoneum and mesentery: A clinicopathologic analysis of 44 cases. J Surg Oncol 1985;28:177-86.  Back to cited text no. 9
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10.
Wile AG, Evans HL, Romsdahl MM. Leiomyosarcoma of soft tissue: A clinicopathologic study. Cancer 1981;48:1022-32.  Back to cited text no. 10
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11.
Hernando E, Charytonowicz E, Dudas ME, Menendez S, Matushansky I, Mills J, et al. The AKT-mTOR pathway plays a critical role in the development of leiomyosarcomas. Nat Med 2007;13:748-53.  Back to cited text no. 11
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12.
Setsu N, Kohashi K, Endo M, Yamamoto H, Tamiya S, Takahashi Y, et al. Phosphorylation of signal transducer and activator of transcription 3 in soft tissue leiomyosarcoma is associated with a better prognosis. Int J Cancer 2013;132:109-15.  Back to cited text no. 12
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13.
Abraham JA, Weaver MJ, Hornick JL, Zurakowski D, Ready JE. Outcomes and prognostic factors for a consecutive case series of 115 patients with somatic leiomyosarcoma. J Bone Joint Surg Am 2012;94:736-44.  Back to cited text no. 13
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14.
Enneking WF, Spanier SS, Goodman MA. A system for the surgical staging of musculoskeletal sarcoma. Clin Orthop 1980;153:106-20.  Back to cited text no. 14
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15.
Le QT, Fu KK, Kroll S, Fitts L, Massullo V, Ferrell L, et al. Prognostic factors in adult soft-tissue sarcomas of the head and neck. Int J Radiat Oncol Biol Phys 1997;37:975-84.  Back to cited text no. 15
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16.
Konomoto T, Fukuda T, Hayashi K, Kumazawa J, Tsuneyoshi M. Leiomyosarcoma in soft tissue: Examination of p53 status and cell proliferating factors in different locations. Hum Pathol 1998;29:74-81.  Back to cited text no. 16
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17.
Strander H, Turesson I, Cavallin-Ståhl E. A systematic overview of radiation therapy effects in soft tissue sarcomas. Acta Oncol 2003;42:516-31.  Back to cited text no. 17
    


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