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Journal of Bone and Soft Tissue Tumors (JBST) is the official Journal of The Indian Musculo Skeletal Oncology Society
Desmoplastic Fibroma of the Distal Femur in a Young Man: A Rare Case Report
Original Article | Volume 6 | Issue 3 | JBST September – December 2020 | Page 2-4 | Suresh Babu, Abhishek Vaish, Raju Vaishya DOI: 10.13107/jbst.2020.v06i03.30
Author: Suresh Babu[1], Abhishek Vaish[1], Raju Vaishya[1]
[1]Department of Orthopaedics and Joint Replacement Surgery, Indrparastha Apollo Hospitals, New Delhi, India.
Address of Correspondence
Dr. Suresh Babu,
Department of Orthopaedics and Joint Replacement Surgery, Indrparastha Apollo Hospitals, Sarita Vihar, New Delhi – 110 076, India.
E-mail: yssureshbabu@gmail.com
Introduction: Lytic lesions in the distal femur in a mature skeleton though a common presentation for various tumors, desmoplastic fibroma (DFB) of bone is a rare occurrence. Review of the literature shows its incidence of 0.06% to 0.3%. In the majority of reported cases, the diagnosis has been obtained on histopathological examination. Treatment varies from aggressive curettage to amputations. We describe a novel surgical technique for dealing such lesions.
Case Report: A 24-year-old male presented with swelling around with DFB the left distal femur presented with pain for 4 months which progressed in severity and led to inability to bear weight. On clinical examination, he had a tender discrete swelling over the medial aspect of the left distal femur. Radiographic examination showed an eccentric lytic lesion in the metaphyseoepiphyseal region of the left distal femur. An extended curettage using phenol (10%) as adjuvant therapy was performed. The cavity was packed with bone cement and the distal femur was fixed by spanning the lytic lesion with a distal femoral locking plate. At months follow-up, he reported complete resolution of symptoms, and on examination, he had pain free and full range of motion, without any signs of recurrence.
Conclusion: An effort should be made to obtain a pre-operative histopathological diagnosis of the tumor type. In cases of equivocal findings, a diagnosis of DFB of the bone should be considered. Extended curettage, phenol ablation, and bone cement with plate augmentation offer an effective treatment modality in the treatment of DFB.
Keywords: Desmoplastic fibroma, benign bone tumors, surgery, knee, femur.
Reference:
1. Campanacci M. Desmoid fibroma. In: Bone and Soft Tissue Tumors: Clinical Features, Imaging, Pathology and Treatment. Wien: Springer-Verlag; 1999. p. 143-8.
2. Campanacci L. Desmoid fibroma. In: Diagnosis of Musculoskeletal Tumors and Tumor-like Conditions. Berlin, Germany: Springer; 2019. p. 61-3.
3. Mazabraud A. Desmoid fibroma. In: Pathology of bone tumours. Springer, Berlin, Heidelberg; 1998;14:167-72 https://doi.org/10.1007/978-3-642-95839-7_14.
4. Kalil RK. Desmoplastic fibroma of bone. In: Tumors and Tumor-Like Lesions of Bone. Berlin, Germany: Springer; 2020. p. 451-7.
5. Inwards CY, Unni KK, Beabout JW, Sim FH. Desmoplastic fibroma of bone. Cancer 1991;68:1978-83.
6. Gebhardt MC, Campbell CJ, Schiller AL, Mankin HJ. Desmoplastic fibroma of bone: A report of eight cases and review of the literature. J Bone Joint Surg Am 1985;67:732-47.
7. Gao S, Cai Q, Yao W, Wang J, Zhang P, Wang X. Desmoplastic (collagenous) fibroma of the femur: A case report and review of the literature. Oncol Lett 2013;6:1285-8.
8. Gong LH, Liu WF, Ding Y, Geng YH, Sun XQ, Huang XY. Diagnosis and differential diagnosis of desmoplastic fibroblastoma by clinical, radiological, and histopathological analyses. Chin Med J 2018;131:32-6.
9. Crim JR, Gold RH, Mirra JM, Eckardt JJ, Bassett LW. Desmoplastic fibroma of bone: Radiographic analysis. Radiology 1989;172:827-32.
10. Xu Y, Wang Y, Yan J, Bai X, Xing G. Desmoplastic fibroma of the femur with atypical image findings: A case report. Medicine 2018;97:e13787.
11. Kang HS, Ahn JM, Kang Y. Radiographic findings. In: Oncologic Imaging: Bone Tumors. Berlin, Germany: Springer; 2017. p. 273-307.
12. Tanwar YS, Kharbanda Y, Rastogi R, Singh R. Desmoplastic fibroma of bone: A case series and review of literature. Indian J Surg Oncol 2018;9:585-91.
13. Nishida J, Tajima K, Abe M, Honda M, Inomata Y, Shimamura T, et al. Desmoplastic fibroma. Aggressive curettage as a surgical alternative for treatment. Clin Orthop Relat Res 1995;320:142-8.
14. Taconis WK, Schütte HE, van der Heul RO. Desmoplastic fibroma of bone: A report of 18 cases. Skeletal Radiol 1994;23:283-8.
15. Bohm P, Krober S, Greschniok A, Laniado M, Kaiserling E. Desmoplastic fibroma of the bone. A report of two patients, review of the literature, and therapeutic implications. Cancer 1996;78:1011-23.
16. Takazawa K, Tsuchiya H, Yamamoto N, Nonomura A, Suzuki M, Taki J, et al. Osteosarcoma arising from desmoplastic fibroma treated 16 years earlier: A case report. J Orthop Sci 2003;8:864-8.
17. Abdelwahab IF, Klein MJ, Hermann G, Steiner GC, Yang DC. Osteosarcoma arising in a desmoplastic fibroma of the proximal tibia. AJR Am J Roentgenol 2002;178:613-5.
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Editorial May – August 2020
Editorial | Volume 6 | Issue 2 | JBST May – August 2020 | Page 1 | Yogesh Panchwagh, Ashish Gulia, Ashok Shyam. 10.13107/jbst.2020.v06.i02.20
Author:Dr. Yogesh Panchwagh[1], Dr. Ashish Gulia[2] & Dr. Ashok Shyam[3],[4]
[1]Orthopaedic Oncology Clinic, Pune, India.
[2]Orthopedic Oncology Services, Department of Surgical Oncology,
Tata Memorial Hospital, Mumbai.
[3]Indian Orthopaedic Research Group, Thane, India
[4]Sancheti Institute for Orthopaedics &Rehabilitation, Pune, India
Address of Correspondence
Dr. Yogesh Panchwagh.
Orthopaedic Oncology Clinic, 101, Vasant plot 29, Bharat Kunj
Society -2, Erandwana, Pune – 38, India.
Email: drpanchwagh@gmail.com
In the last 4 months, we have seen the real impact of Covid-19 pandemic in India with multiple lockdowns implemented nationwide. With all offices and businesses, barring the healthcare sector and allied units, coming to a standstill or switching to work-from-home format, there have been major changes to the ways in which we used to accomplish things.
Certain branches of medicine and especially some hospital units saw tremendous surge in work pressure given the intense demand of workforce required to handle the pandemic. Many others faced challenges in form of reduced patient load affecting the economics and had to fight daunting circumstances to stay afloat. Everyone had to adapt to face the situation and emerge out less scathed if not unscathed.
However, the situation presented a unique opportunity to all. It ranged from data accrual and documentation pertaining to the various aspects of pandemic in case of some, while for others who had free time, it helped in analysis and manuscript writing of pending publication work. Everyone had an opportunity to contribute to research and hence we have seen a surge in manuscript submissions and publications. Thankfully, the review process for almost all remained mostly unaffected and hence the turnaround times for article publication were not affected significantly.
Adaptability remains the key for the human race. We need to look for the brighter aspects through the ongoing mayhem, fight the doubtful thoughts lurking around, take advantage of whatever situation we may be forced into and focus on the work that needs to be done.
JBST team is proud and happy to present this issue on time despite multiple challenges. Kudos to all the contributors who have shown grit and a strong will to fight on.
Dr. Yogesh Panchwagh
Dr. Ashish Gulia
Dr. Ashok Shyam
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The Baby Bump and Malignant Lump: A Case Report of a Pregnant Patient with Pelvic Sarcoma
Original Article | Volume 6 | Issue 2 | JBST May-August 2020 | Page 25-27 | Daniela Kristina D. Carolino, Mary Rose C. Gonzales, Richard S. Rotor. DOI: 10.13107/jbst.2020.v06i02.28
Author: Daniela Kristina D. Carolino[1], Mary Rose C. Gonzales[1], Richard S. Rotor[2]
[1]Department of Orthopaedics, Institute of Orthopedics and Sports Medicine, St. Luke’s Medical Center, Quezon City, Metro Manila, Philippines.
[2]Department of Musculoskeletal tumors, Institute of Orthopedics and Sports Medicine, St. Luke’s Medical Center, Quezon City, Metro Manila, Philippines.
Address of Correspondence
Dr. Daniela Kristina D. Carolino, 279 E. Rodriguez Sr. Ave, Quezon City 1112 Metro Manila.
E-mail: dkdcarolino@gmail.com
Abstract
Purpose: The occurrence of a malignancy during the course of pregnancy is uncommon but devastating. Due to the relative rarity of the condition, guidelines for management are largely based on small retrospective studies or case series with limited follow-up. With this case, we aim to discuss the options of management and rationalize the decisions, in which the course of treatment of this patient has proceeded.
Materials and Methods: We describe the clinical presentation of the patient as well as the radiological findings, diagnostic tests, and management during the course of the disease.
Results: A 26-year-old (gravida 2, para 1) Filipino female presented initially with a limp and gradual enlargement of a mass on the right hip while with a single intrauterine pregnancy of 8–9 weeks age of gestation. As the growth the mass continued, the patient sought consult at our institution at 22 weeks gestation and was presented with options for management. The patient opted for conservative treatment with chemotherapy over hemipelvectomy. The neonate was eventually delivered at 35 weeks of gestation and the demise of the patient ensued 2 months postpartum.
Conclusion: The treatment of malignancy in the pregnant patient should be individualized and largely dependent on the decision of the mother, once full disclosure of all options of management, possible risks, and prognosis has been discussed.
Reference:
1. Zagouri F, Dimitrakakis C, Marinopoulos S, Tsigginou A, Dimopoulos MA. Cancer in pregnancy: Disentangling treatment modalities. ESMO Open 2016;1:e000016.
2. Peccatori FA, Azim HA, Orecchia R, Hoekstra HJ, Pavlidis N, Kesic V, et al. Cancer, pregnancy and fertility: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Ann Oncol 2013;24 Suppl 6:vi160-70.
3. Postl LK, Gradl G, von Eisenhart-Rothe R, Toepfer A, Pohlig F, Burgkart R, et al. Management of musculoskeletal tumors during pregnancy: A retrospective study. BMC Womens Health 2015;15:48.
4. Zarkavelis G, Petrakis D, Fotopoulos G, Mitrou S, Pavlidisa N. Bone and soft tissue sarcomas during pregnancy: A narrative review of the literature. J Adv Res 2016;7:581-7.
5. Figueiro-Filho EA, Al-Sum H, Parrish J, Wunder JS, Maxwell C. Maternal and fetal outcomes in pregnancies affected by bone and soft tissue tumors. AJP Rep 2018;8:e343-8.
6. Rimawi BH, Green V, Lindsay M. Fetal implications of diagnostic radiation exposure during pregnancy: Evidence-based recommendations. Clin Obstet Gynecol 2016;59:412-8.
7. Kal HB, Struikmans H. Radiotherapy during pregnancy: Fact and fiction. Lancet Oncol 2005;6:328-33.
8. Azim HA, Peccatori FA, Pavlidis N. Treatment of the pregnant mother with cancer: A systemic review on the use of cytotoxic, endocrine, targeted agents and immunotherapy during pregnancy. Part I: Solid tumors. Cancer Treat Rev 2010;36:101-9.
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A Clinicopathological Study of Ewing’s Sarcoma/PNET experience from a Tertiary Cancer Centre in North East India
Original Article | Volume 6 | Issue 2 | JBST May-August 2020 | Page 21-24 | Jagannath Dev Sharma, Argha Baruah, Anupam Sarma, Lopa Mudra Kakoti, Nizara Baishya, Shiraj Ahmed. DOI: 10.13107/jbst.2020.v06i02.27
Author: Jagannath Dev Sharma[1], Argha Baruah[1], Anupam Sarma[1], Lopa Mudra Kakoti[1], Nizara Baishya[1], [2], Shiraj Ahmed[1]
[1]Department of Pathology, Dr.B.Borooah Cancer Institute, Guwahati, Assam, India.
[2]Department of Hospital based Cancer registry, Dr.B. Borooah Cancer Institute, Guwahati, Assam.
Address of Correspondence
Dr. Argha Baruah,
Department of Pathology, Dr.B.Borooah Cancer Institute,Guwahati-781028, Assam, India.
E-mail: argha20@gmail.com
Abstract
Introduction: Ewing sarcoma (ES)/PNET is an aggressive malignant tumor with small round cell morphology affecting mainly children and adolescents. The aim of this study was to study the clinicopathological parameters and immunohistochemical panel of skeletal and extraskeletal ES and to correlate with overall survival.
Case Report: Medical files of 70 patients with ES treated at our center between 2009 and 2015 were retrospectively evaluated. The clinico pathological parameters were extracted and statistically correlated with overall survival(OS). Among 70cases of ES ,41 cases were males and 29 cases were females. Most common age group was 10–20 years. Skeletal involvement was seen in 45 cases(64.2%) and 25cases (35.8%) were extraskeletal. The most common skeletal sites of involvement was lower extremity involving the Femur (24%) and the most common extraskeletal site involved in our study was sinonasal area(5.7%), followed by chestwall, thigh, orbital, calf, gluteal, kidney, and vulva. Two cases showed involvement of the central nervous system(CNS) involving pineal gland and the ventricle. Two cases showed multiple sites of involvement both including chest wall and thigh. Twenty-nine cases(41.4%) showed metastasized disease. The most common site of metastasis was lung followed by bone and brain. Recurrence was seen in 14 cases(20%). Overall 5-year survival was 24%. There was statistically significant correlation found between tumor size (≥8cm) and 5year survival. Furthermore, significant correlation was found between metastasis and 5-year survival.
Conclusion: ES is an aggressive tumor involving skeletal and extraskeletal sites affecting commonly young people, with a poor prognosis for patients with maximum diameter ≥8cm. Metastasisis common in ES and is also a poor prognostic factor.
Keywords: Ewing’sarcoma, skeletal, extraskeletal, survival, metastasis.
Reference:
1. Paulssen M, Ahrens S, Dunst J, Winkelmann W, Exner GU, Kotz R, et al. Localized Ewing tumor of bone: Final results of the cooperative Ewing’s sarcoma study CESS 86. J Clin Oncol 2001;19:1818-29.
2. Ewing J. Diffuse endothelioma of bone. Proc N Y Path Soc 1921;7:17-24.
3. Angervall L, Enzinger FM. Extraskeletal neoplasm resembling Ewing’s sarcoma. J Cancer 1975;36:240-51.
4. Askin FB, Rosai J, Sibley RK, Dehner LP, McAlister WH. Malignant small cell tumor of the thoracopulmonary region in childhood: A distinctive clinicopathologic entity of uncertain histogenesis. J Cancer 1979;43:2438-51.
5. Bellan DG, Filho RJ, Garcia JG, de Toledo Petrilli M, Viola DC, Schoedl MF, et al. Ewing’s sarcoma: Epidemiology and prognosis for patients treated at the pediatric oncology institute, IOP-GRAACC-UNIFESP. Rev Bras Ortop 2015;47:446-50.
6. Akhavan A, Binesh F, Shamshiri H, Ghanadi F. Survival of patients with Ewing’s sarcoma in Yazd-Iran. Asian Pac J Cancer Prev 2014;15:4861-4.
7. Biswas B, Rastogi S, Khan SA, Mohanti BK, Sharma DN, Sharma MC, et al. Outcomes and prognostic factors for Ewing-family tumors of the extremities. J Bone Joint Surg Am 2014;96:841-9.
8. Lee JA, Kim DH, Lim JS, Koh JS, Kim MS, Kong CB, et al. Soft-tissue Ewing sarcoma in a low-incidence population: Comparison to skeletal Ewing sarcoma for clinical characteristics and treatment outcome. Jpn J Clin Oncol 2010;40:1060-7.
9. Worch J, Matthay KK, Neuhaus J, Goldsby R, DuBois SG. Ethnic and racial differences in patients with Ewing sarcoma. J Cancer 2010;116:983-8.
10. Louati S, Senhaji N, Chbani L, Bennis S. EWSR1 rearrangement and CD99 expression as diagnostic biomarkers for Ewing/PNET sarcomas in a Moroccan population. Dis Markers 2018;2018:7971019.
11. Folpe AL, Hill CE, Parham DM, O’Shea PA, Weiss SW. Immunohistochemical detection of FLI-1 protein expression: A study of 132 round cell tumors with emphasis on CD99-positive mimics of Ewing’s sarcoma/primitive neuroectodermal tumor. Am J Surg Pathol 2000;24:1657-62.
12. Rossi S, Orvieto E, Furlanetto A, Laurino L, Ninfo V, Dei Tos AP. Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody. Mod Pathol 2004;17:547-52.
13. Ahmed SH, Rahman NA, Meng L. Cytokeratin immunoreactivity in Ewing sarcoma/primitive neuroectodermal tumour. Malays J Pathol 2013;35:139-45.
14. Lucas DR, Bentley G, Dan ME, Tabaczka P, Poulik JM, Mott MP. Ewing sarcoma vs lymphoblastic lymphoma. A comparative immunohistochemical study. Am J Clin Pathol 2001;115:11-7.
15. Machado I, Noguera R, Pellin A, Lopez-Guerrero JA, Piqueras M, Navarro S, et al. Molecular diagnosis of Ewing sarcoma family of tumors: A comparative analysis of 560 cases with FISH and RT-PCR. Diagn Mol Pathol 2009;18:189-99.
16. Tirode F, Surdez D, Ma X, Parker M, Le Deley MC, Bahrami A, et al. Genomic landscape of Ewing sarcoma defines an aggressive subtype with co-association of STAG2 and TP53 mutations. Cancer Discov 2015;4:1342-53.
17. Stahl M, Ranft A, Paulussen M, Bölling T, Vieth V, Bielack S, et al. Risk of recurrence and survival after relapse in patients with Ewing sarcoma. Pediatr Blood Cancer 2011;57:549-53.
18. Obata H, Ueda T, Kawai A, Ishii T, Ozaki T, Abe S, et al. Clinical outcome of patients with Ewing sarcoma family of tumors of bone in Japan: The Japanese musculoskeletal oncology group cooperative study. J Cancer 2007;109:767-75.
19. Oksuz DC, Tural D, Dincbas FO, Dervisoglu S, Turna H, Hiz M, et al. Non-metastatic Ewing’s sarcoma family of tumors of bone in adolescents and adults: Prognostic factors and clinical outcome-single institution results. Tumor J 2014;100:452-8.
20. El Weshi A, Allam A, Ajarim D, Al Dayel F, Pant R, Bazarbashi S, et al. Extraskeletal Ewing’s sarcoma family of tumours in adults: Analysis of 57 patients from a single institution. Clin Oncol 2010;22:374-81.
21. Pradhan A, Grimer RJ, Spooner D, Peake D, Carter SR, Tillman RM, et al. Oncological outcomes of patients with Ewing’s sarcoma: Is there a difference between skeletal and extra-skeletal Ewing’s sarcoma? J Bone Joint Surg Br 2011;93:531-6.
22. Bosma SE, Ayu O, Dijkstra PD, Fiocco M, Gelderblom H. Prognostic factors for survival in Ewing sarcoma: A systematic review. Surg Oncol 2018;27:603-10.
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Osteosarcoma of the Rib: A Case Report
Original Article | Volume 6 | Issue 2 | JBST May-August 2020 | Page 17-20 | Kanu Priya Bhatia, Sameer Rastogi, Ekta Dhamija, Adarsh Barwad, Nishant Bhatia, Jyoutishman Saika. DOI: 10.13107/jbst.2020.v06i02.26
Author: Kanu Priya Bhatia[1], Sameer Rastogi[1], Ekta Dhamija[2], Adarsh Barwad[3], Nishant Bhatia[4], Jyoutishman Saika[5]
[1]Department of Medical Oncology, Dr. B.R Ambedkar Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India,
[2]Department of Radiodiagnosis, Dr. B.R Ambedkar Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India,
[3]Department of Pathology, All India Institute of Medical Sciences, New Delhi, India,
[4]Department of Orthopaedics, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India,
[5]Department of Surgical Oncology, All India Institute of Medical Sciences, New Delhi, India.
Address of Correspondence
Dr. Kanu Priya Bhatia,
Department of Medical Oncology, Dr. B.R Ambedkar Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India.
E-mail: bhatiakanu13@gmail.com
Abstract
Introduction: Osteosarcoma (OS) is the most common primary malignancy of bone in children and adolescents. OS has a predilection for the metaphyseal region of the long bones. The most common site of involvement is the distal femur followed by proximal tibia, proximal humerus, middle and proximal femur, and other bones. Primary OS of chest wall is a very rare entity.
Case Report: We, here, describe a case of a 14-year-old boy who presented to our center with a chest wall swelling and pleural effusion, which was subsequently diagnosed as chest wall OS originating from the rib. We treated him with neoadjuvant chemotherapy (Ifosfamide, Adriamycin, and Carboplatin). He showed an excellent transient response to the chemotherapy, after which he underwent an en bloc resection of the tumor. He was then started on adjuvant chemotherapy, but unfortunately, he relapsed soon after the last cycle and later on succumbed to the disease.
Conclusion: Chest wall OS is an infrequent malignancy. Pathological diagnosis is difficult and requires a high index of suspicion. Data regarding the prognostic factors are scarce, and no concrete guidelines are available for the management of such patients.
Keywords: Chest wall, osteosarcoma, rib.
Reference:
1. Moghazy K, Al-Jehani Y, El-Baz A, El-Ghoneimy Y. Incidental finding of a large chest wall osteosarcoma–a case report. Gulf J Oncolog 2007;1:93-7.
2. Sabatier R, Bouvier C, de Pinieux G, Sarran A, Brenot-Rossi I, Pedeutour F, et al. Low-grade extraskeletal osteosarcoma of the chest wall: Case report and review of literature. BMC Cancer 2010;10:645.
3. Qian J, Zhang XY, Gu P, Shao JC, Han BH, Wang HM. Primary thoracic extraskeletal osteosarcoma: A case report and literature review. J Thorac Dis 2017;9:E1088-95.
4. Bathurst N, Sanerkin N. Osteoclast-rich osteosarcoma. Br J Radiol 1986;59:667-73.
5. Chow LT. Giant cell rich osteosarcoma revisited-diagnostic criteria and histopathologic patterns, Ki67, CDK4, and MDM2 expression, changes in response to bisphosphonate and denosumab treatment. Virchows Arch 2016;468:741-55.
6. Daw N, Neel M, Rao B, Billups C, Wu J, Jenkins J, et al. Frontline treatment of localized osteosarcoma without methotrexate. Cancer 2011;117:2770-8.
7. Baez JC, Lee EY, Restrepo R, Eisenberg RL. Chest wall lesions in children. Am J Roentgenol 2013;200:W402-19.
8. Burt M, Fulton M, Wessner-Dunlap S, Karpeh M, Huvos AG, Bains MS, et al. Primary bony and cartilaginous sarcomas of chest wall: Results of therapy. Ann Thorac Surg 1992;54:226-32.
9. Ikeda H, Takeo M, Kayata H, Mikami R, Nakamoto Y, Yamamoto M. A case of rapidly growing osteosarcoma of the rib. Ann Thorac Cardiovasc Surg 2014;20:521-4.
10. Inchara Y, Crasta J, Ananthamurthy A, Mohanty S. An unusual case of primary osteosarcoma of the rib in an adult. Indian J Med Paediatr Oncol 2010;31:18.
11. Rad MP, Masoum SF, Layegh P, Rad MS. Primary osteosarcoma of the sternum: A case report and review of the literature. Arch Bone Joint Surg 2014;2:272-5.
12. Lim W, Sarji SA, Yik Y, Ramanujam T. Osteosarcoma of the rib. Biomed Imaging Interv J 2008;4:e7.
13. Masoud S. Scapula osteosarcoma. Biomed J Sci Tech Res 2017;1:739-42.
14. Bielack SS, Kempf-Bielack B, Delling G, Exner GU, Flege S, Helmke K, et al. Prognostic factors in high-grade osteosarcoma of the extremities or trunk: An analysis of 1,702 patients treated on neoadjuvant cooperative osteosarcoma study group protocols. J Clin Oncol 2002;20:776-90.
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Retrospective Study of Seven Patients with Tumoral Calcinosis
Original Article | Volume 6 | Issue 2 | JBST May-August 2020 | Page 12-16 | Kshitij Manerikar, Abhijeet Salunke, Jaymin V. Shah, Mayur Kamani, Shashank Pandya. DOI: 10.13107/jbst.2020.v06i02.25
Author: Kshitij Manerikar[1], Abhijeet Salunke[1], Jaymin V. Shah[1], Mayur Kamani[1], Shashank Pandya[1]
[1]Department of Surgical Oncology, Gujarat Cancer Research and Institute, Ahmedabad, Gujarat, India.
Address of Correspondence
Dr. Kshitij Manerikar,
A-302, Divyadeep, Ram Mandir Road, TPS-3, Borivali West, Mumbai – 400 092, Maharashtra, India.
E-mail: drkshitijmanerikar@gmail.com
Abstract
Introduction: Calcium deposition in the skin has been termed as calcinosis cutis. Tumoral calcinosis is idiopathic form of calcinosis cutis. Etiology of idiopathic calcinosis cutis is unknown. It is characterized by periarticular deposition of amorphous calcium salts around large joints. Our diligent search through literature could not find any consensus on the etiopathogenesis and treatment modalities for tumoral calcinosis.
Materials and Methods: A retrospective study of seven patients of tumoral calcinosis treated with complete surgical excision over a period of 1 year was done. Demographic details were compiled. Routine blood investigations were performed. All patients underwent radiographs and magnetic resonance imaging (MRI) scans of involved part. We did not perform computed tomography (CT) or bone scan in any of our patients. All seven patients underwent surgery and were followed up till 2 years.
Results: In our study, five were female and two were male patients ranging from 31 to 76 years. Size of swelling varied from 2 to 15 cm. Most common location was hip. Serum calcium, phosphorus, and alkaline phosphatase were normal in all patients. Radiographs showed well-outlined periarticular cluster of calcifications in the soft tissues around joint. MRI revealed round to oval multiple cystic lesions around the affected region, but not involving the joint.
Conclusion: Tumoral calcinosis is always the diagnosis of exclusion. It can be normophosphatemic or hypophosphatemic subtype. Large joints are more commonly affected. One can rely on radiographs for diagnosis. MRI for knowing exact location of lesion, its relationship with adjacent structures and planning of surgery is advocated. Complete surgical excision is the only optimum treatment of tumoral calcinosis.
Keywords: Amorphous calcium phosphate, hyperphosphatemia, X-ray film, hip joint, calcinosis, magnetic resonance imaging.
Reference:
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3. Chaabane S, Chelli-Bouaziz M, Jelassi H, Mrad K, Smida M, Ladeb MF. Idiopathic tumoral calcinosis. Acta Orthop Belg 2008;74:837-45.
4. Muddegowda P, Lingegowda J, Ramachandrarao R, Konapur PG. Calcinosis cutis: Report of 4 Cases. J Lab Physicians 2011;3:125-6.
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7. Smack D, Norton S, Fitzpatrick J. Proposal for a pathogenesis-based classification of tumoral calcinosis. Int J Dermatol 1996;35:265-71.
8. Noyez J, Murphree S, Chen K. Tumoral calcinosis, a clinical report of eleven cases. Acta Orthop Belg 1993;59:49-54.
9. Aprin H, Sinha A. Tumoral calcinosis: Report of a case in a one year old child. Clin Orthop 1984;185:83-6.
10. Niall D, Fogarty E, Dowling F, Moore D. Spontaneous regression of tumoral calcinosis in an infant: A case report. J Pediatr Surg 1998;33:1429-31.
11. Fujii T, Matsui N, Yamamoto T, Yoshiya S, Kurosaka M. Solitary intra-articular tumoral calcinosis of the knee. Arthroscopy 2003;19:E1.
12. Bittmann S, Gunther M, Ulus H. Tumoral calcinosis of the gluteal region in a child: Case report with overview of different soft-tissue calcifications. J Pediatr Surg 2003;38:E4-7.
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