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Journal of Bone and Soft Tissue Tumors (JBST) is the official Journal of The Indian Musculo Skeletal Oncology Society
Clear Cell Sarcoma: A Rare Aggressive Tumor with Potential Diagnostic Challenge
Vol 4 | Issue 2 | July-Dec 2018 | Page 30-32 | Yash B Rabari, Nikhil Suri, D.V. Prasad, Siddharth Shah.
Authors: Yash B Rabari [1], Nikhil Suri [1], D.V. Prasad [1], Siddharth Shah [1].
[1] Department of Orthopaedics, Rural Medical Collage, LoniBk, Maharashtra, India.
Address of Correspondence
Dr. Yash B. Rabari.
107, NRI Hostel, PMT Campus, LoniBk, Tal.-Rahta, Dist.- Ahmadnagar Maharastra 413736
Email: dryash.rabari@gmail.com
Abstract
Clear cell sarcoma (CCS) is an exceedingly rare tumor of young adults with melanocytic differentiation. The exact incidence is largely unknown, although occasional case series mention CCS comprising less than 1% of all soft tissue sarcoma. CCS is a deep-seated tumor, typically involving tendons and aponeuroses. It has a predilection for lower extremities, particularly around the foot and ankle region, accounting for nearly 40% of cases. A primary dermal origin is rarer. We report a case of primary cutaneous CCS of 42 year old female located on the left popliteal fossa. Complete excision led to relief of symptoms
Keywords: Clear cell Sarcoma, Tumor, CCS
References
1. Dim DC, Cooley LD, Miranda RN. Clear cell sarcoma of tendons and aponeuroses: A review. Arch Pathol Lab Med. 2007;131:152–6.
2. Deenik W, Mooi WJ, Rutgers EJ, Peterse JL, Hart AA, Kroon BB. Clear cell sarcoma (malignant melanoma) of soft parts: A clinicopathological study of 30 cases. Cancer. 1999;86:969–75.
3. Kazakos CJ, Galanis VG, Giatromanolaki A, Verettas DA, Sivridis E. Clear cell sarcoma of the scapula. A case report and review of literature. World J SurgOncol. 2006;4:48–53. [PMC free article]
4. Hantschke M, Mentzel T, Rutten A, Palmedo G, Calonje E, Lazar AJ, et al. Cutaneous clear cell sarcoma: A clinicopathological, immunohistochemical, and molecular analysis of 12 cases emphasizing its distinction form dermal melanoma. Am J SurgPathol. 2010;34:216–22.
5. Hisaoka M, Ishida T, Kuo TT, Matsuyama A, Imamura T, Nishida K, et al. Clear cell sarcoma of soft tissue: A clinicopathological, immunohistochemical, and molecular analysis of 33 cases. Am J SurgPathol. 2008;32:452–60.
6. Patel RM, Downs-Kelly E, Weiss SW, Folpe AL, Tubbs RR, Tuthill RJ, et al. Dual-color, break-apart fluorescence in situ hybridization for EWS gene rearrangement distinguishes clear cell sarcoma of soft tissue from malignant melanoma. Mod Pathol. 2005;18:1585–90.
7. Rodriguez-Martin M, Saez- Rodriguez M, Esquivel B, Gonzalez RS, Cabrera AN, Herrera AM. Clear cell sarcoma: A case mimicking primary cutaneous malignant melanoma. Indian J Dermatol. 2009;54:168–72.
8. Enzinger FM. Clear cell sarcoma of tendons and aponeuroses: An analysis of 21 cases. Cancer. 1965;18:1163–74.
9. Chung EB, Enzinger FM. Malignant melanoma of soft parts.A reassessment of clear cell sarcoma. Am J SurgPathol. 1983;7:405–13.
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Inter-Osseous Epidermoid Inclusion Cyst Of The Sacrum: A Case Report
Vol 4 | Issue 2 | July-Dec 2018 | Page 27-29 | Mike Parry, Anish Patel, Rajesh Botchu, Vaiyapuri S, Desai, Jeys Lee.
Authors: Mike Parry [1], Anish Patel [2], Rajesh Botchu [2], Vaiyapuri S [3], Desai [4], Jeys Lee [1].
[1] Department of Orthopedic Oncology.
[2] Department of Musculoskeletal Radiology,
[3] Department of pathology,The Royal Orthopedic Hospital, Bristol Road South, Northfield, Birmingham, UK
[4] Department of Surgery, The Queen Elizabeth Hospital, Birmingham, UK
Address of Correspondence
Dr. Rajesh Botchu
Department of Musculoskeletal Radiology, The Royal Orthopedic Hospital, Bristol Road South, Northfield, Birmingham, UK
Email: drbrajesh@yahoo.com
Abstract
Whilst common in subcutaneous locations, inclusion epidermoid cysts arising from the pelvic bones are extremely rare. We report the case of a patient presenting with coccydynia and altered bowel habit who, following investigation was found to have a large cystic lesion of the sacrum with extending anteriorly into the presacral tissue resulting in compression of the rectum. The lesion was treated by surgical resection, sacrificing the lower sacral nerve roots. Histology confirmed an epidermoid cyst. Whilst a rare presenting symptom, careful assessment of the patient presenting with coccydynia and altered bowel function must raise the suspicion of a sacral pathology that demands further investigation.
Keywords: sacral epidermoid cyst, rare presentation
References
1. Handa U, Kumar S, Mohan H. Aspiration cytology of epidermoid cyst of terminal phalanx. Diagn Cytopathol. 2002;26(4):266–7.
2. Adachi H, Yoshida H, Yumoto T, Naniwa S, Okuno M, Morimoto K, et al. Intraosseous epidermal cyst of the sacrum. A case report. Acta Pathol Jpn. 1988;38(12):1561–4.
3. Muellner T, Nicolic A, Lang S, Schabus R, Kwasny O. Epidermoid cyst after arthroscopic knee surgery. Arthroscopy. 1998;14(3):331–4.
4. Ettinger RL, Manderson RD. Implantation keratinizing epidermoid cysts. A review and case history. Oral Surg Oral Med Oral Pathol. 1973;36(2):225–30.
5. Musharrafieh RS, Tawil AN, Saghieh SS, Macari G, Atiyeh BS. Epidermoid cyst of the thumb. Orthopedics. 2002;25(8):862–3.
6. Zadek I, Cohen HG. Epidermoid cyst of the terminal phalanx of a finger; with a review of the literature. Am J Surg. 1953;85(6):771–4.
7. Vion-Dury J, Vincentelli F, Jiddane M, Van Bunnen Y, Rumeau C, Grisoli F, et al. MR imaging of epidermoid cysts. Neuroradiology. 1987;29(4):333–8.
8. Epstein WL, Kligman AM. Epithelial cysts in buried human skin. AMA Arch Derm. 1957;76(4):437–45.
9. Jaiswal A, Shetty AP, Rajasekaran S. Precoccygeal epidermal inclusion cyst presenting as coccygodynia. Singapore Med J. 2008;49(8):e212–4.
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Osseous Hydatid Disease: Mimicker of primary bone neoplasms.: Report of 2 cases
Vol 4 | Issue 2 | July-Dec 2018 | Page 25-26 | Sujit Joshi, Yogesh Panchwagh.
Authors: Sujit Joshi [1], Yogesh Panchwagh [2].
[1] Dept. of Pathology Deenanath Mangeshkar Hospital and Research Centre, Pune
[2] Orthopaedic Oncology Clinic, Pune, India.
Address of Correspondence
Dr. Sujit Joshi,
Dept. of Pathology Deenanath Mangeshkar Hospital and Research Centre, Pune
Email:sujitjoshi30@gmail.com
Abstract
Osseous hydatid disease is rare, and it is even rare to see it in extremities or pelvis. At times these lesions present as destructive lesions on radiographs and mimic neoplasm. Here we present two such cases, one arising in the lower end of femur mimicking chondrosarcoma and required excision and surgical stabilization. Second case presented as a cystic lesion in iliac bone suggestive of aneurysmal bone cyst. Both are quite unusual presentation and hydatid disease should always be kept in differential while dealing with such lesion
Keywords: Osseous hydatid disease, chondrosarcoma, aneurysmal bone cyst
References
1. International Journal of Research in Medical Sciences; Kumar R et al. Int J Res Med Sci. 2017 Mar;5(3):1126-1130.
2. M Laubscher et al SA Orthopedic Journal Winter 2018, Page 44-48.
3. Dahniya MH, Hanna RM, Ashebu S, Muhtaseb SA, el-Beltagi A, Badr S, et al. The imaging appearances of hydatid disease at some unusual sites. Br J Radiol. 2001;74:283-9.
4. Mattern CJ, Gardner MJ, Grose A, Helfet DL. Echinococcus disease of the bone presenting as a femoral shaft non-union. ClinOrthopRelat Res. 2007;458:220-5.
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Periosteal Chondroma of Radius Diaphysis – Rare Presentation
Vol 4 | Issue 2 | July-Dec 2018 | Page 23-24 | Praveen Kumar Pandey, Raaghav Rai Verma.
Authors: Praveen Kumar Pandey [1], Raaghav Rai Verma [2].
[1] Esi-pgimsr model hospital basaidarapur new Delhi – 110015 , GGSIPU university
[2] Department of orthopaedics, Dr RML Hospital, new Delhi
Address of Correspondence
Dr. Praveen Pandey,
ESI-pgimsr Model Hospital Basaidarapur new Delhi – 110015 , GGSIPU university, India
Email: praveen2gir@yahoo.co.in
Abstract
Periosteal chondroma is a rare bone tumor. This is a slow growing benign cartilaginous tumor of bone. It is rarely reported in literature among Asian population. A 35 years old female presented to orthopaedics OPD with dull aching pain and mild swelling over volar aspect right forearm. Patient advised to undergo radiographs and MRI which showed the evidence of periosteal chondroma over anteromedial aspect right radius diaphysis. Hereby we are reporting our case of periosteal chondroma of radius- a rare pathology at rare location. Patient was operated with marginal excision and showed no recurrence during 2 year follow up.
Keywords: periosteal chondroma; rare; radius; asian; marginal excision.
References
1. Keiller VH. Cartilaginous tumors of bone. Surg Gynecol Obstet 1925; 40: 510.
2. Lichtenstein L, Hall J. Periosteal chondroma: a distinctive benign cartilage tumor. J Bone Joint Surg Am 1952; 34-A: 691-7.
3. Boriani S, Bacchini P, Bertoni F, Campanacci M. Periosteal chondroma. A review of twenty cases. J Bone Joint Surg Am 1983; 65: 205-12.
4. deSantos LA, Spjut HJ. Periosteal chondroma: a radiographic spectrum. Skeletal Radiol 1981; 6: 15-20.
5. Nojima T, Unni KK, McLeod RA, Pritchard DJ. Periosteal chondroma and periosteal chondrosarcoma Am J Surg Pathol 1985; 9: 666-77.
6. Sinha S, Singhania GK, Campbell AC. Periosteal chondroma of the distal radius. J Hand Surg [Br ] 1999; 24: 747-9.
7. Lisanti M, Buongiorno L, Bonicoli E, Cantini G. Periosteal chondroma of the proximal radius: a case report. Chir Organi Mov. 2005 Oct-Dec;90(4):403-7.
8. Parodi KK, Farrett W, Paden MH, Stone PA. A report of a rare phalangeal periosteal chondroma of the foot. J Foot Ankle Surg. 2011 Jan-Feb;50(1):122-5.
9. Al-Qudah AS, Abu-Ali HM, Al-Hussaini MA, Massad IM. Periosteal chondroma of the clavicle: case report and review of the literature. Int J Surg. 2009 Apr;7(2):140-1.
10. Sirichai Luevitoonvechkij , Olarn Arphornchayanon, Taninnit Leerapun , Songsak Khunsree. Periosteal Chondroma of the Proximal Humerus: A Case Report and Review of the Literature J Med Assoc Thai 2006;89(11): 1970-75.
11. Woertler K, Blasius S, Brinkschmidt C, Hillmann A, Link TM, Heindel W. Periosteal chondroma: MR characteristics. J Comput Assist Tomogr 2001; 25: 425-30.
12. Lewis MM, Kenan S, Yabut SM, Norman A, Steiner G. Periosteal chondroma. A report of ten cases and review of the literature. Clin Orthop Relat Res 1990; 185-92.
13. Bauer TW, Dorfman HD, Latham JT Jr. Periosteal chondroma. A clinicopathologic study of 23 cases. Am J Surg Pathol 1982; 6: 631-7.
14. Nosanchuk JS, Kaufer H. Recurrent periosteal chondroma. Report of two cases and a review of the literature. J Bone Joint Surg Am 1969; 51: 375-80.
15. Rockwell MA, Saiter ET, Enneking WF. Periosteal chondroma. J Bone Joint Surg Am 1972; 54: 102-8.
16. Bertoni F, Boriani S, Laus M, Campanacci M. Periosteal chondrosarcoma and periosteal osteosarcoma. Two distinct entities. J Bone Joint Surg Br 1982; 64: 370-6.
17. Lorente MF, Bonete Lluch DJ, Marti PV. Childhood periosteal chondroma. Arch Orthop Trauma Surg 2000; 120: 605-8.
18. Mora R, Guerreschi F, Fedeli A, Alfarano M, Angi V. Two cases of periosteal chondroma. Acta Orthop Scand 1988; 59: 723-7.
19. Ricca RL Jr, Kuklo TR, Shawen SB, Vick DJ, Schaefer RA. Periosteal chondroma of the cuboid presenting in a 7-year-old-boy. Foot Ankle Int 2000; 21: 145-9.
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Glomangioma of the Tibia- A rare presentation
Vol 4 | Issue 2 | July-Dec 2018 | Page 20-22 | Sijin K S, Bhaskar Subin Sugath, Vijaya Mohan S, Vinod Kumar P, Nanda Kachare.
Authors: Sijin K S [1], Bhaskar Subin Sugath [1], Vijaya Mohan S [1], Vinod Kumar P [1], Nanda Kachare [1].
[1] Dept of Aster Orthopaedics, Aster Medcity, Cochin, India
Address of Correspondence
Dr. Bhaskar Subin Sugath,
Consultant, Aster Pathology, Aster Medcity, Cochin, India
Email: bhaskarsubin@gmail.com
Abstract
A 35yr old female patient presented with a pulsatile swelling of right proximal tibia present for 2 years. There was a rapid increase in size and pain in the 6 weeks preceding presentation. Radiographs showed a lytic lesion of about 8cm x 8cm in the anteromedial aspect of the proximal tibia with MRI showing a highly vascular lesion. A needle biopsy and histopathological examination confirmed the diagnosis of Glomangioma. It was treated with marginal resection of the tumour and fibular grafting of the defect.
References
1. Shugart RR, Soule EH, Johnson EW Jr. Glomus tumor. Surg Gynecol Obstet, 1963; 117:334–340.
2. Schoen FJ, Cotran RS. The blood vessels. In: Kumar V, Cotran RS, Robins SL, editors. Robins Basic Pathology. Philadelphia: Saunders, 2003;325-36
3. Bhaskaranand K, Navadgi BC. Glomus tumour of the hand. J Hand Surg [Br] 2002;27:229-31.
4. Ozdemir O, Coskunol E, Ozalp T, et al. Glomus tumors of the finger: a report on 60 cases. Acta Orthop Traumatol Turc 2003;37:244-8.
5. Bahk WJ, Mirra JM, Anders KH. Intraosseous glomus tumor of the fibula. Skeletal Radiol 2000;29:708-12.
6. Settakorn J, Chalidapong P, Rangdaeng S, et al. Primary intraosseous glomus tumor: a case report. J Med Assoc Thai 2001;84:1641-5.
7. Weiss SW, Goldblum JR. Perivascular tumors. In Soft tissue tumors. 4th edition. pp.985–1035, 2001, Mosby, St Louis.
8. Kransdorf MJ, Murphey MD. Vascular and lymphatic tumors. In Imaging of soft tissue tumors. 2nd edition. pp. 150–188, 2006, Lippincott Williams & Wilkins, Philadelphia.
9. Rozmaryn LM, Sadler AH, Dorfman HD. Intraosseous glomus tumor in the ulna: a case report. Clin Orthop, 1987; 220: 126–129.
10. Urakawa H, Nakashima H, Yamada Y et al. Intraosseous glomus tumor of the ulna: A case report with radiographic findings and a review of the literature Nagoya J. Med. Sci. 70. 127 ~ 133, 2008.
11. Bessho Y, Kataoka O, Sho T, Kitazawa S, Okada S. Intraosseous glomus tumor in the upper thoracic spine complicating compression myelopathy: a case report. Spine, 1991; 16: 988–990.
12. Bjorkengren AG, Resnick D, Haghighi P, Sartoris DJ. Intraosseous glomus tumor: report of a case and review of the literature. AJR, 1986; 147: 739–741.
13. Chan CW. Intraosseous glomus tumor: case report. J Hand Surg, 1981; 6A: 368–369.
14. Johnson DL, Kuschner SH, Lane CS. Intraosseous glomus tumor of the phalanx: a case report. J Hand Surg, 1993; 18A: 1026–1028.
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16. Mackenzie DH. Intraosseous glomus tumours. Report of two cases. J Bone Joint Surg, 1962; 44B: 648–651.
17. Payer M, Grob D, Benini A, Varga S, Hodler J, Martin JB. Intraosseous glomus tumor of the thoracic spine. Case illustration. J Neurosurg, 2002; 96: 137.
18. Robinson JC, Kilpatrick SE, Kelly DL Jr. Intraosseous glomus tumor of the spine: case report and review of the literature. J Neurosurg, 1996; 85: 344–347.
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Biphasic synovial sarcoma – A ten year experience with molecular profile and clinical outcome
Vol 4 | Issue 2 | July-Dec 2018 | Page 14-19 | Kala Gnanasekaran Kiruthiga, Anne Jennifer Prabhu, Rekha Pai, Sramana Mukhopadhyay, Reka.K, V.T.K.Titus, Selvamani Backianathan.
Authors: Kala Gnanasekaran Kiruthiga [1], Anne Jennifer Prabhu [1], Rekha Pai [1], Sramana Mukhopadhyay [1], Reka.K [2], V.T.K.Titus [3], Selvamani Backianathan [4].
[1] Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India.
[2] Department of Biostatistics, Christian Medical College, Vellore, Tamil Nadu, India.
[3] Department of Orthopaedics, Christian Medical College, Vellore, Tamil Nadu, India.
[4] Department of Radiotherapy, Christian Medical College, Vellore, Tamil Nadu, India.
Address of Correspondence
Dr. Anne Jennifer Prabhu,
Address: Department of Pathology, ASHA Building, Christian Medical College, Vellore, Tamil Nadu, India.
E-mail: annejennifer91@gmail.com
Abstract
Context: Synovial sarcoma is one of the commonly encountered spindle cell sarcomas of soft tissue. However, Biphasic synovial sarcoma (BSS) is a rare subtype of synovial sarcoma with limited literature on clinical profile, molecular characteristics and survival outcome.
Aims: We propose to describe the immuno – morphology, clinical features, molecular profile and outcome of patients with BSS.
Settings and Design: This retrospective study included 13 cases of BSS, 3.2% of all synovial sarcomas diagnosed over 10 years in our institute. The clinico-pathological features were studied in detail and immunohistochemistry for TLE-1, EMA, CD34, CD99 and S100 was done. Real time PCR and DNA sequencing for the common translocations (SYT-SSX1/ SYT-SSX2) were performed.
Statistical analysis used: Statistical Package for Social Services (SPSS) software Version 21.0 (Armonk, NY: IBM Corp).
Results: BSS was most commonly seen in young, the most common site being soft tissue of extremities (92.3%). 53.8% of patients presented at Enneking stage IIB. The FNCLCC grade varied between 2(46.2%) and 3(53.8%). All cases were positive for EMA and TLE-1; negative for CD34 and S100. Ten of the eleven (90.9%) patients tested had SYT-SSX1 translocation. Over a period of 8 to 46 months, 53.8% cases were alive and well with no evidence of disease; three had (30%) recurred, one (10%) had lung metastasis and one (10%) died.
Conclusions: BSS is most common in extremities. The immunohistochemical profile matches that of monophasic synovial sarcoma. FNCLCC grade is 2 to 3; however the grade does not correlate with clinical outcome. Most cases show SYT-SSX1 translocation. 53.8% cases were alive and well after a mean follow up of 20 months.
Keywords: Biphasic, follow-up, immunoprofile, SYT-SSX1, synovial sarcoma.
Keyword: Biphasic synovial sarcoma has the same immunoprofile as the monophasic subtype. All patients who tested positive had SYT-SSX1 translocation. More than 50% of patients were alive and well after 20 months.
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