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Skeletal Muscle Metastases Arising from Renal Cell Carcinoma in a 58-Year-Old Male: A Case Report

Original Article | Volume 6 | Issue 3 | JBST September – December 2020 | Page 20-25 | Sybill Sue M. Moser, Edwin Joseph R. Guerzon, John Christopher R. Ragasa DOI: 10.13107/jbst.2020.v06i03.035

Author: Sybill Sue M. Moser[1], Edwin Joseph R. Guerzon[1], John Christopher R. Ragasa[1]

[1]Department of Orthopedics, Institute of Orthopedics and Sports Medicine, St. Luke’s Medical Center – Quezon City, Manila, Philippines.

Address of Correspondence:
Dr. Sybill Sue M. Moser
2632 Molave St. United Hills Village,
Parañaque City, Metro Manila, Philippines.
E-mail: sybillsuemoser@gmail.com


Introduction: Renal cell carcinoma (RCC) is the most common malignant kidney tumor, commonly metastasizing to the lung, lymph nodes, bones, and brain. Here, we present a rare case of renal cell skeletal muscle metastases (SMMs), accounting for only <1% of all RCC metastases.
Methods: This is a descriptive report on the clinical course, diagnostic investigations, and surgical treatment of a case of SMM in a patient previously diagnosed with RCC.
Results: This is a 58-year-old male who previously underwent radical nephrectomy for RCC, presenting with a 5-month history of a rapidly enlarging left gluteal mass. The mass was confirmed to be renal clear cell metastasis through percutaneous biopsy. On magnetic resonance imaging, two heterogeneously enhancing lesions in the left gluteal muscle and right paralumbar muscles at the level of L4 and L5 were noted. Positron emitted tomography scan confirmed no other metastases. He underwent wide excision of the right paraspinal mass and buttockectomy for the left gluteal mass.
Conclusion: SMM in RCC is rare, thus tissue diagnosis and imaging is deemed necessary to rule out any other primary sarcoma. In these cases, patients may benefit from metastasectomy. Regular follow-up and surveillance are recommended for these patients to rule out recurrence.
Keywords: Renal cell carcinoma, metastases, extraskeletal, buttockectomy.


Reference:

1. Graves A, Hessamodini H, Wong G, Lim WH. Metastatic renal cell carcinoma: Update on epidemiology, genetics, and therapeutic modalities. ImmunoTargets Ther 2013;2:73-90.
2. Sakamoto A, Yoshia T, Matsuura S, Tanaka K, Matsuda S, Oda Y, et al. Metastasis to the gluteus maximus muscle from renal cell carcinoma with special emphasis on MRI features. World J Surg Oncol 2007;5:88.
3. Surov A, Hainz M, Holzhausen HJ, Arnold D, Katzer M, Schmidt J, et al. Skeletal muscle metastases: Primary tumours, prevalence, and radiological features. Eur J Radiol 2010;20:649-58.
4. Sountoulides P, Metaxa L, Cindolo L. Atypical presentations and rare metastatic sites of renal cell carcinoma: A review of case reports. J Med Case Rep 2011;5:429.
5. Ramchandani P. Post-treatment surveillance of renal cancer renal cell. In: Patel U, editor. Carcinoma of the Kidney. 1st ed. Cambridge: Cambridge University Press; 2008. p. 185-202.
6. Goger Y, Piskin M, Balasar M, Kilinc M. Unusual Presentation of Renal Cell Carcinoma: Gluteal Metastasis. Case Reports in Urology; 2013.
7. Haygood TM, Sayyouh M, Wong J, Lin J, Matamoros A, Sandler C, et al. Skeletal muscle metastasis from renal cell carcinoma. Sultan Qaboos Univ Med J 2015;15:327-37.
8. Crispen PL, Blute ML. Role of cytoreductive nephrectomy in the era of targeted therapy for renal cell carcinoma. Curr Urol Rep 2012;13:38-46.


How to Cite this article: Goyal AK, Vaish A, Vaishya R, Baweja P| A Rare Recurrent Chondromyxoid Fibroma of the Proximal Tibia in an Adolescent Female | Journal of Bone and Soft Tissue Tumors | September-December 2020; 6(3): 20-25.

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A Rare Recurrent Chondromyxoid Fibroma of the Proximal Tibia in an Adolescent Female

Original Article | Volume 6 | Issue 3 | JBST September – December 2020 | Page 16-19 | Ashish Kumar Goyal, Abhishek Vaish, Raju Vaishya, Pankaj Baweja DOI: 10.13107/jbst.2020.v06i03.34

Author: Ashish Kumar Goyal[1], Abhishek Vaish[1], Raju Vaishya[1], Pankaj Baweja[2]

[1]Department of Orthopaedics and Joint Replacement Surgery, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi, India.
[2]Department of Pathology, Indraprastha Apollo Hospital, Sarita Vihar, New Delhi, India.

Address of Correspondence
Dr. Ashish Kumar Goyal, Department of Orthopaedics and Joint Replacement Surgery, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi – 110 076, India.
E-mail: drgoyal41@gmail.com


Introduction: Lytic lesions of the bone are often seen in children and young adults. These are usually benign but sometimes are locally aggressive bone tumors. Lesser known causes of lytic lesions are chondromyxoid fibroma (CMF) and its recurrence is very rare. The accepted treatment for CMF is surgical curettage with bone grafting and the review of the literature shows that recurrence rate is 3–22%.
Case Report: We present a rare case of an adolescent girl of 14 years who presented with a recurrent bone tumor of the proximal tibia, after 4 years of the primary curettage. It turned out to be a CMF. She presented with swelling over the right upper leg for the past month. It was gradual in onset, progressive, and non-radiating in nature. There was no history of trauma. She had had a similar complaint 4 years back in 2016, for which she was operated elsewhere, where curettage and synthetic bone substitutes were used for filling the bone cavity. She was taken up for surgery for extended curettage and bone cementing. The histopathological examination was suggestive of CMF. Subsequent follow-up, she reported complete resolution of symptoms with a pain-free range of knee movements and no radiological signs of recurrence.
Conclusion: The accepted treatment for CMF is surgical curettage with bone grafting, but the recurrence rate is 3–22%.
Keywords: Chondromyxoid fibroma, benign lesions, tibia, recurrence.


Reference:
1. Berg E, Sokolay M. Diagnosis and management of lytic lesions in bone. JAMA 1982;247:2099.
2. Canale ST, Beaty JH, Campbell WC. Campbell’s Operative Orthopaedics. St. Louis, MO: Mosby; 2012. p. 860-4.
3. Vargas B. Orthopedic Surgery for Fibrous Dysplasia. Background, Pathophysiology, Etiology. New York: Medscape; 2019. Available from: https://www.emedicine.medscape.com/article/1255262-overview. [Last accessed on 2020 Aug 22].
4. Klenke FM, Wenger DE, Inwards CY, Rose PS, Sim FH. Giant cell tumor of bone: Risk factors for recurrence. Clin Orthop Relat Res 2010;469:591-9.
5. Bhamra JS, Al-Khateeb H, Dhinsa BS, Gikas PD, Tirabosco R, Pollock RC, et al. Chondromyxoid fibroma management: A single institution experience of 22 cases. World J Surg Oncol 2014;12:283.
6. Vaishya R, Agarwal A, Gupta N, Vijay V. Fibrocartilaginous dysplasia of the bone: A rare variant of fibrous dysplasia. Cureus 2016;8:e448.
7. Hahn SB, Kim SH, Cho NH, Choi CJ, Kim BS, Kang HJ. Treatment of osteofibrous dysplasia and associated lesions. Yonsei Med J 2007;48:502-10.
8. Rui J, Guan W, Gu Y, Lao J. Treatment and functional result of desmoplastic fibroma with repeated recurrences in the forearm: A case report. Oncol Lett 2015;11:1506-8.
9. Hasan O, Ali M, Mustafa M, Ali A, Umer M. Treatment and recurrence of giant cell tumors of bone-a retrospective cohort from a developing country. Ann Med Surg (Lond) 2019;48:29-34.
10. Bush JB, Sweeney JP, Robison JE, DeMoss B, Meyer MS. Chondromyxoid fibroma of the radial shaft treated with nonvascularized fibular autograft. Am J Orthop (Belle Mead NJ) 2010;39:30-4.
11. Castle JT, Kernig ML. Chondromyxoid fibroma of the ethmoid sinus. Head Neck Pathol 2011;5:261-4.
12. Fomete B, Adeosun OO, Awelimobor DI, Olayemi L. Chondromyxoid fibroma of the mandible: Case report and review of the literature. Ann Maxillofac Surg 2014;4:78-80.
13. Wu CT, Inwards CY, O’Laughlin S, Rock MG, Beabout JW, Unni KK. Chondromyxoid fibroma of bone: A clinicopathologic review of 278 cases. Hum Pathol 1998;29:438-46.
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18. Vaishya R, Agarwal AK, Vijay V. ‘Salvage treatment’ of aggressive giant cell tumor of bones with denosumab. Cureus 2015;7:e291.


How to Cite this article: Goyal AK, Vaish A, Vaishya R, Baweja P| A Rare Recurrent Chondromyxoid Fibroma of the Proximal Tibia in an Adolescent Female | Journal of Bone and Soft Tissue Tumors | September-December 2020; 6(3): 16-19.

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Role of Intraoperative Nerve Stimulator? In a Case of Ulnar Nerve Schwannoma. A Case Report with Four-Year Follow-up and Review of Literature

Original Article | Volume 6 | Issue 3 | JBST September – December 2020 | Page 13-15 | Kalaivanan Kanniyan, Ying Lee Lam, Ho Wai Yip Kenneth, Yau Raymond DOI: 10.13107/jbst.2020.v06i03.33

Author: Kalaivanan Kanniyan[1],[2], Ying Lee Lam[1], Ho Wai Yip Kenneth[1], Yau Raymond[1]

[1]Department of Orthopaedic Oncology, Queen Mary Hospital, University of Hong Kong, Hong Kong,
[2]Department of Arthroplasty and Oncology, Asian Joint Reconstruction Institute – AJRI at SIMS Hospitals, Chennai, Tamil Nadu, India.

Address of Correspondence
Dr. Kalaivanan Kanniyan,
Division of General Orthopaedics and Oncology, Queen Mary Hospital, University of Hong Kong, Hong Kong.
E-mail: drkkbriyan@gmail.com


Introduction: Schwannoma (neurolimmea) is an uncommon benign nerve sheath tumor arising from the peripheral nerve system with an incidence of 5%. They are slow growing tumors affecting all age group and occur in isolation. They are common in upper extremity followed by the lower extremity.
Case report: She presented with complaint of pain and swelling over her right wrist disturbing her daily activities such as eating and holding her walker. The swelling was sudden on onset slowly progressive in nature over many years. She was on treatment for diabetes mellitus, hypertension, and Parkinson. She had left leg weakness due to polio and hence walker bound. She underwent occipitocervical fusion for cervical vertebrae C1 and C2 subluxation and cervical myelopathy. The swelling was 2.5 cm × 2.5 cm, soft-tissue mass over the volar aspect of right wrist mobile only in the longitudinal direction. Hand grip was fair with sensory loss along the ulnar nerve distribution. There was no hypothenar muscle wasting. Magnetic resonance imaging suggested of neurogenic tumor of ulnar nerve origin. Patient was planned for enucleation (excision biopsy). The anesthesia nerve block device was used as nerve stimulator to identify the motor nerve and preserved it. Enucleation was not possible and hence the tumor was removed with a segment of sensory fibers. Histology finding was consistent with schwannoma. Postoperatively, the patient was pain free and able to hold the walker and carry on her daily activities. At 4 year follow-up the patient had no recurrence of such tumor or any other complication in the operated side.
Conclusion: Enucleation is the treatment of choice. Patient age, complaints, comorbidities, functional demand, tumor location, and intraoperative findings with nerve stimulator help in deciding the choice of nerve grafting along with enucleation.
Keywords: Schwannoma, ulnar nerve, enucleation, sural nerve graft, intraoperative nerve stimulator.


Reference:
1. Forthman CL, Blazar PE. Nerve tumors of the hand and upper extremity. Hand Clin 2004;20:233-42.
2. Trãistaru R, Enãchescu V, Manu CD, Gruia C, Ghiluşi M. Multiple right schwannoma. Rom J Morphol Embryol 2008;49:235-9.
3. Ozdemir O, Ozsoy MH, Kurt C, Coskunol E, Calli I. Schwannomas of the hand and wrist: Long term results and review of the literature. J Ortho Surg (Hong Kong) 2005;13:267-72.
4. Chris YK, Fung B, Fok M, Zhu J. Schwannoma in the upper limbs. BioMed Res Int 2013;2013:167196.
5. Donner TR, Voorhies RM, Kline DG. Neural sheath tumors of major nerves. J Neurosurg 1994;81:362-73.
6. White NB. Neurilemomas of the extremities. J Bone Joint Surg Am 1967;49:1605-10.
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9. Beaman FD, Kransdorf MJ, Menke DM. Schwan-noma: Radiologic-pathologic correlation. Radiographics 2004;24:1477-81.
10. Van Herendael B, Heyman S, Schepper A, Gielen J, Parizel PM. Schwannoma of left ulnar nerve. JBR BTR 2006;89:156-7.


How to Cite this article: Kanniyan K, Lam YL, Kenneth HWY, Raymond Y | Role of Intraoperative Nerve Stimulator? In a Case of Ulnar Nerve Schwannoma. A Case Report with Four-Year Follow-up and Review of Literature | Journal of Bone and Soft Tissue Tumors | Sep-Dec 2020; 6(3): 13-15.

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Effect of Combination Chemotherapy and Radiotherapy in the Management of a Pathological Fracture in High-grade Osteosarcoma with Limb Salvage Procedure – A Case Report

Original Article | Volume 6 | Issue 3 | JBST September – December 2020 | Page 9-12 | V.R.Ganesan, T.C.Prem Kumar, Sanjay.C, Roopesh Kumar.S DOI: 10.13107/jbst.2020.v06i03.32

Author: V.R.Ganesan[1], T.C.Prem Kumar[1], Sanjay.C[1], Roopesh Kumar.S[1]

[1]Department of Orthopaedic Surgery & Traumatology,
Madurai Medical College & GRH, Madurai, Tamilnadu. India.

Address of Correspondence
Dr. V R Ganesan,
Department of Orthopaedic Surgery & Traumatology,
Madurai Medical College & GRH , Madurai, Tamilnadu. India.
E-mail: sphospital@hotmail.com


Introduction: Osteosarcoma (OS), the most common primary bone tumor, is known to be relatively a radioresistant tumor. Pathological fracture in OS denotes its aggressive biological response and so it was considered a contraindication to limb salvage in earlier days. Radiotherapy has its role only in cases that are inoperable or have poor prognostic factors. In recent years, there have been major advances in the management of pathological fractures in high-grade OS (HGOS). This case report is about the effect of combination chemotherapy and radiotherapy in the management of pathological fracture in HGOS with limb salvage procedure.
Case Report: A 19-year-old male with pain and swelling in his right lower third of thigh and inability to walk for 3 months was diagnosed as a case of OS right distal femur with a pathological fracture. Open biopsy was done which confirmed the diagnosis as HGOS, staged Enneking IIb. He was treated with a combination of chemotherapy, external beam radiotherapy, tumor resection, and modular resection prosthesis.
Results: There were no immediate, early, and late complications. At the end of 1½ years, his functional recovery is good and he has reached more than 70% of the right knee functions. He has no signs of recurrence at present. He has got a better quality of life and functional activity with the prosthesis compared to what an amputated limb can produce.
Conclusion: Pathological fracture in OS is not a contraindication to limb salvage. Radiotherapy can be used in combination with chemotherapy and limb salvage surgery in HGOS with a pathological fracture. This combination treatment helps in increasing the chances of limb sparing surgery with good local control and tumor necrosis rate. The new knowledge that radiotherapy can be effective when used with chemotherapy has shown good result in our case.
Keywords: Osteosarcoma, pathological fracture, external beam radiotherapy, limb salvage, custom modular prosthesis.


Reference:
1. Abudu A, Sferopoulos NK, Tillman RM, Carter SR, Grimer RJ. The surgical treatment and outcome of pathological fractures in localized osteosarcoma. J Bone Joint Surg Br 1996;78:694-8.
2. Ferguson PC, McLaughlin CE, Griffin AM, Bell RS, Deheshi BM, Wunder JS. Clinical and functional outcomes of patients with a pathologic fracture in high-grade osteosarcoma. J Surg Oncol 2010;102:120-4.
3. Scully SP, Ghert MA, Zurakowski D, Thompson RC, Gebhardt MC. Pathologic fracture in osteosarcoma: Prognostic importance and treatment implications. J Bone Joint Surg Am 2002;84:49-57.
4. Glasser DB, Lane JM, Huvos AG, Marcove RC, Rosen G. Survival, prognosis and therapeutic response in osteogenic sarcoma: The Memorial Hospital experience. Cancer 1992;69:698-708.
5. Finn HA, Simon MA. Limb-salvage surgery in the treatment of osteosarcoma in skeletally immature individuals. Clin Orthop Relat Res 1991;262:108-18.
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7. Bacci G, Ferrari S, Longhi A, Donati D, Manfrini M, Giacomini S, et al. Nonmetastatic osteosarcoma of the extremity with pathologic fracture at presentation: Local and systemic control by amputation or limb salvage after preoperative chemotherapy. Acta Orthop Scand 2003;74:449 54.
8. Kim MS, Lee SY, Lee TR, Cho WH, Song WS, Cho SH, et al. Prognostic effect of pathologic fracture in localized osteosarcoma: A cohort/case controlled study at a single institute. J Surg Oncol 2009;100:233 9.
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10. Longhi A, Errani C, De Paolis M, Mercuri M, Bacci G. Primary bone osteosarcoma in the pediatric age: State of the art. Cancer Treat Rev 2006;32:423-36.
11. Jeon DG, Lee SY, Kim JW. Bone primary sarcomas undergone unplanned intralesional procedures the possibility of limb salvage and their oncologic results. J Surg Oncol 2006;94:592-8.
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13. Ebeid W, Amin S, Abdelmegid A. Limb salvage management of pathologic fractures of primary malignant bone tumors. Cancer Control 2005;12:57-61.
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15. Cui Q, Li DF, Liu C, Guo J, Liu SB, Liu YS, et al. Two case-reports of the limb salvage treatment of osteosarcoma consolidated with obvious pathological fractures. Pathol Oncol Res 2011;17:973-9.
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18. Yin K, Liao Q, Zhong D, Ding J, Niu B, Long Q, et al. Metaanalysis of limb salvage versusamputation for treating high grade and localized osteosarcoma in patients with pathological fracture. Exp Ther Med 2012;4:889-94.
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20. Machak GN, Tkachev SI, Solovyev YN, Sinyukov PA, Ivanov SM, Kochergina NV, et al. Neoadjuvant chemotherapy and local radiotherapy for high-grade osteosarcoma of the extremities. Mayo Clin Proc 2003;78:147-55.
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22. Dincbas FO, Koca S, Mandel NM, Hiz M, Dervişoğlu S, Seçmezacar H, et al. The role of preoperative radiotherapy in non-metastatic high-grade osteosarcoma of the extremities for limb-sparing surgery. Int J Radiat Oncol Biol Phys 2005;62:820-8.


How to Cite this article: Ganesan VR, Kumar TCP, Sanjay C, Kumar SR| Effect of Combination Chemotherapy and Radiotherapy in the Management of a Pathological Fracture in High-grade Osteosarcoma with Limb Salvage Procedure – A Case Report | Journal of Bone and Soft Tissue Tumors | September-December 2020; 6(3): 9-12.

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Langerhans Cell Histiocytosis of the Spine in a Child: A Rare Case and a Diagnostic Dilemma

Original Article | Volume 6 | Issue 3 | JBST September – December 2020 | Page 5-8 | Abigail R Tud, Jose Bayani O Aliling, Carlo Emmanuel J SumpaicoDOI: 10.13107/jbst.2020.v06i03.31

Author: Abigail R Tud[1], Jose Bayani O Aliling[1], Carlo Emmanuel J Sumpaico[1]

[1]Department of Orthopedics, The Medical City, Ortigas Avenue, Pasig City, Metro Manila 1605, Philippines.

Address of Correspondence
Dr. Abigail R Tud,
Unit 5F Tower 1, Sonata Private Residences, Lourdes corner St. Francis Avenues, East Wack Wack, Mandaluyong City, Metro Manila 1505, Philippines.
E-mail address: abitud@gmail.com


Introduction: Langerhans cell histiocytosis (LCH) comprises a rare spectrum of disorders characterized by abnormal proliferation of histiocytes. Lesions may be limited to a single system, or present as disseminated disease, with subsequent worse prognosis. Radiologic findings for spinal LCH are non-specific however and must be carefully differentiated from Pott’s disease (spinal tuberculosis, or [TB]) in endemic countries.
Case Report: An 8-year-old female was brought for consult due to back pain and compression deformity on plain radiographs, unaccompanied by constitutional symptoms. Computed tomography (CT) scan of the thoracic spine was done, which showed osteolysis of the T11 vertebra and paravertebral soft-tissue extension. Findings were deemed suggestive of tuberculous spondylitis, and CT-guided biopsy was performed for confirmation. Acid-fast Bacilli smears as well as Gram stains were negative. While waiting for definitive results, the patient was started on an empiric treatment regimen for TB. Final tissue and fluid cultures were negative for Mycobacterium tuberculosis, as were Gene Xpert and TB polymerase chain reaction studies. Histopathologic analysis showed atypical mononuclear histiocytes surrounded by inflammatory cells, suggestive of LCH. At present, the patient is 2 years post-biopsy, with stable lesions and no evidence of multi-system involvement.
Conclusion: In the setting of a spinal lesion in a child with a benign clinical history and no other pertinent laboratory findings, histopathologic analysis constitutes the gold standard in differentiating non-specific features of LCH from spinal TB. Both conditions necessitate long-term follow-up, due to the risk of progression, deformities, and neurologic sequelae.
Keywords: Langerhans cell histiocytosis, pediatric spine, Pott’s disease, tuberculosis, vertebra plana.


Reference:
1. Imbach P, Kühne T, Arceci RJ. Histiocytoses. In: Pediatric Oncology: A Comprehensive Guide. United States: Springer Science and Business Media; 2011.
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How to Cite this article: Tud AR, Aliling JBO, Sumpaico CEJ | Langerhans Cell Histiocytosis of the Spine in a Child: A Rare Case and a Diagnostic Dilemma. | Journal of Bone and Soft Tissue Tumors | September-December 2020; 6(3): 5-8.

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