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  1. Int J Clin Exp Med 2018;11(4):4252-4256 www.ijcem.com /ISSN:1940-5901/IJCEM0066853 Case Report A unique case of upper cervical spinal dural fusocellular sarcoma in a 25-year-old male: a case report and comprehensive treatment analysis Yu-Long Lan1,2,3,4, Shuang Zou3, Xun Wang1, Jin-Shan Xing1, Jia-Cheng Lou1, Hong-Jin Wang4, Bo Zhang1 Departments of 1Neurosurgery, 4Neurology, The Second Affiliated Hospital of Dalian Medical University, Dalian 116023, Liaoning, China; 2Department of Physiology, Dalian Medical University, Dalian 116044, Liaoning, China; 3Department of Neurology, The First Affiliated Hospital of Dalian Medical University, Dalian 116011, Liaoning, China Received March 11, 2017; Accepted February 14, 2018; Epub April 15, 2018; Published April 30, 2018 Abstract: Fusocellular sarcoma is a rare malignant tumor of mesenchymal cells, and a case of fusocellular sarcoma originating from the dura mater spinalis has never been reported before. The high degree of malignancy, undefined histological characteristics and poor clinical prognosis indicate the need for more clinical research of this disease. We hereby present a case of upper cervical spinal dural fusocellular sarcoma. The patient initially appeared to have a meningioma but was eventually diagnosed with fusocellular sarcoma originating from the dura mater spinalis. Despite receiving surgical management and chemotherapy promptly after the diagnosis and undergoing close ob- servation following treatment, the patient still developed metastases to multiple sites of the lung and brain. Taking into account the similar presentation of this rare disease to other entities, the early and accurate diagnosis of cervi- cal spinal dural fusocellular sarcoma is vital. The condition should be considered a life-threatening condition with the possibility of metastasis to other sites of the body. Keywords: Fusocellular sarcoma, dura mater spinalis, diagnosis, therapy Introduction Case presentation Fusocellular sarcoma is a rare type of malig- nant tumor [1], and a case of fusocellular sar- coma originating from the dura mater spinalis has never been reported before. The surpris- ingly high degree of malignancy regarding its clinical manifestation indicates the need for more clinical research of this disease [2, 3]. We hereby present a case of upper cervical spinal dural fusocellular sarcoma. The patient initially appeared to have a meningioma but was even- tually diagnosed with fusocellular sarcoma originating from the dura mater spinalis. Des- pite receiving surgical management and che- motherapy promptly after the diagnosis and undergoing close observation following treat- ment, the patient still developed metastases to multiple sites of the lung and brain. The diag- nostic and therapeutic challenges strongly emphasize the importance of this topic for fu- ture investigations. A 25-year-old man was admitted for a five- month history of discomfort in the neck, a 15-day history of limb weakness, and sudden paralysis for one day. Five months ago, cervical discomfort appeared with no obvious cause; symptoms manifested mainly as cervical stiff- ness and paroxysmal symptoms with each attack lasting for a different length of time. Then, the patient underwent a large cervical vascular color Doppler ultrasound examination at a local hospital. The results indicated occlu- sion of the left internal carotid artery. Four months ago, he was again admitted to the hos- pital with a diagnosis of arteritis. At the hospi- tal, he received hormone therapy and treat- ment to improve circulation as well as various other symptomatic treatments. However, there was still no significant improvement in his symp- toms. Pulmonary computed tomography (CT), cranial CT and magnetic resonance imaging

  2. A case of upper cervical spinal dural fusocellular sarcoma cervical segment of the spinal cord was blocked, forming a cupped mouth shape (Figure 1A, 1B). An abnormal signal shadow with a shuttle shape appeared outside the medulla spinalis but inside the canalis spinalis at C4-C5. The mass crossed over the interverte- bral foramen, and compres- sion and displacement of the corresponding spinal cord was apparent. The size of the occupying lesion was approxi- mately 2.5×1.6×0.6 cm. The initial diagnosis was occupy- Figure 1. Radiological findings: Sagittal T1-WI (A) and axial T2-WI (B) of MRI revealed an intraspinal mass with a protrusion into the medullary cavity (ar- row). MRI: magnetic resonance imaging. ing lesions of the cervical spinal cord, with a more detailed diagnosis indicating meningio- mas. Then, we performed surgical resection of the cervical spinal cord occupying lesions using a posterior median approach. When the dura mater spinalis was open, we found that the sub- dural space was filled with pale red granulation tissue. A granulation tissue mass, tightly ad- hered to the medulla spinalis, was apparent on the backside of the corresponding spinal seg- ment. Even light stimulation could cause great pain for the patient. Partial resection was per- formed for a postoperative biopsy. Two indwell- ing drainage tubes were left for postoperative draining after adequate hemostasis. Part of the resected tissue was taken for biopsy (Figure 2). Under a light microscope, tumor cells appeared spindle-shaped and bunchy, indicating an infil- trative tendency. Endochylema was slightly dyed. The nuclei were spindle-shaped and of various sizes, and nuclear division was appar- ent. In addition, remnants of skeletal muscle were present. Thus, the primary diagnosis could be a malignant spindle cell tumor (epi- dural mass). Histopathological examination revealed the following: CD117 (-), CD34 (-), CK (-), Desmin (-), EMA (-), Ki-67 (+10~20%) (-), PR (-), S-100 (+), SMA (+), Vimentin (+), 34βE12 (-), ALK (-), CK5/6 (-), CK7 (-), HBM45 (-), and Melan-A (-). If clinical metastasis could be excluded, meningo-associated fibrosarcoma could be considered a more accurate diagno- sis. However, surprisingly, postoperative re- examination revealed that multiple lesions with nodulous cumulus high-density shadows with obscure boundaries were observed in the pul- monary CT image (Figure 3A, 3B). The larger lesion may have a diameter of approximately Figure 2. Histopathological examination of the re- sected tissue taken for biopsy after the operation. Immunohistochemical analysis further indicated that the tumor was immunopositive for S-100 (+), SMA (+) and Vimentin (+). (MRI) examination were not obviously abnormal at that time. However, fifteen days ago, limb weakness appeared with a gradual deteriora- tion of symptoms, at which point the patient came to our hospital. We learned that the patient had experienced dysuria the night before, and his limb weakness subsequently became gradually more severe. He exhibited poor appetite and weight loss of greater than 20 kg in the past 20 days. Medical examination revealed the following: neck turning and shrug- ging was abnormal; muscle strength testing revealed 2/5 in the bilateral upper extremity; and the bilateral lower extremities were 3-/5 for strength. The only other myelopathic sign was a positive bilateral Babinski sign. An MRI of the spine revealed an obvious lesion of the cer- vical spinal cord with obvious compression against the cervical medulla spinalis. An upper 4253 Int J Clin Exp Med 2018;11(4):4252-4256

  3. A case of upper cervical spinal dural fusocellular sarcoma compression of the mass of the dura mater spinalis aga- inst the spinal marrow form- ed by the cervical spinal dural fusocellular sarcoma was ob- served. A discontinuous fever occurred after the operation, but no clear focus of infection was found. Dexamethasone has been continuously admin- istered since the operation. In addition, rehydration, hemo- stasis and anti-infection treat- ments as well as nutrition Figure 3. Pulmonary CT scan of the axial (A) and coronal positions (B) show- ing the clinical metastasis of the fusocellular sarcoma. CT: computed tomog- raphy. replenishment for the brain and various other symptomatic treatments were used. The pa- tient’s general condition was good in the days following the operation; the patient exhibited stable vital signs, and his muscle strength improved slightly. Then, the patient was trans- ferred to the oncology department for further chemotherapy. Postoperative follow-up reveal- ed that this patient died one month later with systemic deterioration of the whole body, which was likely due to rapid tumor metastasis. Discussion Sarcomas are a heterogeneous group of solid tumors arising from either soft tissues or bone and accounting for approximately 1% of all can- cers in adults [4]. Fusocellular tumors could be of complex morphology and are generally accompanied by an interstitial component. When fusocellular tumors appear in mesenchy- mal tissues, fusocellular sarcoma is formed. Since large variations in morphology and struc- ture can be observed in fusocellular sarcoma, understanding the patients’ information regard- ing clinical symptoms and anamnesis of related diseases could be significantly important for making accurate diagnoses. Histopathological features and immunohistochemical character- istics were indispensably significant for the final histopathological diagnosis. Intriguingly, fusocellular sarcoma is one of the most com- mon types of soft tissue sarcomas in adults, with an occurrence rate of 50%. It can occur in people of any age, and the ratio of affected males to affected females is approximately 1/3. The tumors are usually located in the der- mis and subcutaneous tissues of the extremi- ties, presenting as a nodule or rash externally. However, fusocellular sarcoma originating from Figure 4. Cranial CT showing the occupying lesion of the right pars frontalis under the cranial plate (arrow) together with a soft tissue mass of the left pars pa- rietalis under the scalp. This further indicates tumor metastasis of the fusocellular sarcoma originating from the dura mater spinalis. 2.3 cm. Bilateral pleural effusion and pulmo- nary swelling of the bilateral lower lobe was apparent. In addition, the anterior inferior medi- astinal lymph nodes were slightly enlarged, and this might indicate tumor metastasis. Cranial CT (Figure 4) indicated an occupying lesion of the right pars frontalis under the cranial plate, together with a soft tissue mass of the left pars parietalis under the scalp. Thus, tumor metas- tasis was found to occur in both the lung and the brain, and the final diagnosis was spindle dural fusocellular sarcoma. Furthermore, a postoperative cervical CT of the upper cervical segment further indicated soft tissue swelling at C4-C5, with a patchy high-density shadow inside and an unclear boundary. In addition, spinal canal stenosis and a slight release of 4254 Int J Clin Exp Med 2018;11(4):4252-4256

  4. A case of upper cervical spinal dural fusocellular sarcoma the dura mater spinalis is quite rare, and there have been no other reports of this type in recent years. Additionally, since the histological characteristics were not obvious, further clas- sification of them was difficult. When fusocel- lular sarcoma originating from the dura mater spinalis occurred, it mostly presented with a high degree of malignancy and poor prognosis. tions performed for any excision of soft tissue sarcoma without regard for preoperative imag- ing or the need to remove a margin of normal tissue covering the cancer [7-9]. In addition, pain relief or disappearance could further sup- port the use of preoperative chemotherapy. High-dose chemotherapy could inhibit lung metastasis and systemic micrometastasis of the whole body. Intriguingly, the effect of preop- erative chemotherapy could also partly predict cure rate, which warrants additional research in the future. These treatments should be per- formed as early as possible, since small lesions are more sensitive to chemotherapy than large tumors and larger ones could be more resis- tant. In addition, it is known that the key for pro- longing the survival of patients with pulmonary metastasis is the complete resection of metas- tasis, and neoadjuvant chemotherapy can re- duce and delay the appearance of pulmonary metastasis. Thus, the appropriate chemothera- py could increase the cure rate of this disease. MRI is useful in the diagnosis of cervical spinal dural fusocellular sarcoma, and should be con- sidered the first choice for diagnosing this con- dition. It is important to have an accurate histo- logical diagnosis to determine the treatment plan. Thus, a timely preoperative biopsy is con- siderably important since the diffusion and cancer metastasis of fusocellular sarcoma could occur rapidly and uncontrollably. In this case, we observed that the tumor cell mem- brane was complete and that the tumor edge was clear and smooth; this could easily be mis- diagnosed as meningioma or neurofibroma [5, 6]. However, the pathological diagnosis after operation indicated that this was fusocellular sarcoma, with an unexpected and extremely high degree of malignancy. We concluded that, with only the patient history and imaging fea- tures, a differential diagnosis between cervical spinal dural fusocellular sarcoma and various other spinal tumors, such as meningioma, could be a challenge without the current histo- logical diagnosis. Furthermore, the intraspinal lesions near the intervertebral foramen indi- cated signs of malignancy. Additionally, various other cervical vertebrae and even the thoracic vertebrae exhibited abnormal signal shadows further confirming the severe metastasis of this malignant tumor. Taking into account the similar presentation of this rare disease to other entities, the early and accurate diagnosis of cervical spinal dural fuso- cellular sarcoma is vital, and the condition should be considered a life-threatening condi- tion with the possibility of metastasis to other sites of the body. The possible forthcoming standard treatment could involve a multidisci- plinary approach including surgery, chemother- apy, and even radiation therapy with the par- ticular preferential order determined on a case-by-case basis. Survivorship is approxi- mately 45% at 5 years after treatment [10]. Although the clinical data in this report need to be more concrete, we believe that they could provide critical information for the design of larger and more focused clinical studies that will be necessary to systematically constitute more standard treatments. Given the low inci- dence of this pathology presenting in tumors of the spinal cord and the lack of treatment guide- lines, each patient’s plan should be considered on a case-by-case basis until further studies are performed to determine optimal evidence- based treatments. Regarding the treatments, it might be roughly separated into preoperative chemotherapy (ne- oadjuvant chemotherapy), surgical operation and postoperative chemotherapy together with radiation therapy. Chemotherapy could be an important treatment for cervical spinal dural fusocellular sarcoma. Chemotherapy can im- prove survival rates by inhibiting lung metasta- sis. Furthermore, preoperative chemotherapy leads to the necrosis of tumor cells, promoting decreases in tumor size and in the edema region of the reaction zone and advancing the disappearance of newly formed tumor vessels. This could also reduce the “unplanned exci- sion”, which was introduced to describe opera- Acknowledgements This work is supported by grants from National Natural Science Foundation of China (Nos. 81372714, 81672480), Liaoning Provincial 4255 Int J Clin Exp Med 2018;11(4):4252-4256

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