Unusual presentation of Chordoma in distal radius

Vol 4 | Issue 2 | July-Dec 2018 | Page 33-35 | Subin Sugath, Jayashree, Shrijith MB.

Authors: Subin Sugath [1], Jayashree [1], Shrijith MB [1].

[1] Dept of Regional Cancer Centre , Trivandrum.

Address of Correspondence
Dr. Subin Sugath
Dept of Regional Cancer Centre , Trivandrum,
Email:bhaskarsubin@gmail.com

Abstract

Chordoma is rarely seen in appendicular skeleton. Here we describe a rare case of chordoma occuring in distal radius. Radiograph showed ill defined lytic distal radius, meta diaphyseal lesion. IHC marker for Chordoma Brachyury was done (TMH , Mumbai), which came diffuse strongly positive- confirming the pre op diagnosis of Extra Axial Chordoma. He was treated with Wide resection of the distal radius and one bone forearm wrist arthrodesis
Keywords: Chordoma, extraxial, radius

References

1. Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Ferreira MJ. Chordoma: current concepts, management, and future directions. Lancet Oncol. 2012 Feb;13(2):e69-76.

2. Nielsen GP, Mangham DC, Grimer RJ, Rosenberg AE. Chordoma periphericum: a case report. Am J Surg Pathol 2001;25:263–7.
3. O’donnell P, Tirabosco R, Vujovic S, Bartlett W, Briggs TW, Henderson S, et al. Diagnosing an extraaxial chordoma of the proximal tibia with the help of brachyury, a molecule required for notochordal differentiation. Skeletal Radiol 2007;36:59–65.
4. Tirabosco R, Mangham DC, Rosenberg AE, Vujovic S, Bousdras K, Pizzolitto S, et al. Brachyury expression in extra-axial skeletal and soft tissue chordomas: a marker that distinguishes chordoma from mixed tumour/myoepithelioma/parachordoma in soft tissue. Am J Surg Pathol 2008;32:572–80.
5. van Akkooi AC, van Geel AN, Bessems JH, den Bakker MA. Extra-axial chordoma. J Bone Joint Surg Br 2006;88:1232–4.
6. DiFrancesco LM, Davanzo Castillo CA, Temple WJ. Extra-axial chordoma. Arch Pathol Lab Med 2006; 130:1871–4.
7. Lantos JE, Agaram NP, Healey JH, Hwang S. Recurrent skeletal extra-axial chordoma confirmed with brachyury: imaging features and review of the literature. Skeletal Radiol 2013;42:1451–9
8. Rekhi B. Primary, large extra-axial chordoma in proximal tibia: a rare case report with literature review and diagnostic implications. APMIS. 2016
Mar;124(3):238-42

How to Cite this article: Sugath S, Jayashree, Shrijith MB. Unusual presentation of Chordoma in distal radius. Journal of Bone and Soft Tissue Tumors July-Dec 2018;4(2): 33-35.

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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.

How to Cite this article: Rabari YB, Suri N, Prasad D.V, Shah S. Clear Cell Sarcoma: A Rare Aggressive Tumor with Potential Diagnostic Challenge . Journal of Bone and Soft Tissue Tumors July-Dec 2018;4(2): 30-32.

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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.

How to Cite this article: Parry M, Patel A, Botchu R, Vaiyapuri S, Desai, Jeys L. Interosseous Epidermoid Inclusion Cyst of the Sacrum: A Case Report. Journal of Bone and Soft Tissue Tumors July-Dec 2018;4(2): 27-29.

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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.

How to Cite this article: Joshi S, Panchwagh Y. Osseous Hydatid Disease: Mimicker of primary bone neoplasms.: Report of 2 cases. Journal of Bone and Soft Tissue Tumors July-Dec 2018;4(2): 25-26.

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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.

How to Cite this article: Pandey PK, Verma RR. Periosteal Chondroma of Radius Diaphysis – Rare Presentation. Journal of Bone and Soft Tissue Tumors July-Dec 2018;4(2): 23-24.

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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.
15. Kobayashi Y, Kawaguchi T, Imoto K, Yamamoto T. Intraosseous glomus tumor in the sacrum: a case report. Acta Pathol Jpn, 1990; 40: 856–859.
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.

How to Cite this article: Sijin KS, Sugath BS, Vijaya MS, Vinod KP, Kachare N. Glomangioma of the Tibia- A rare presentation . Journal of Bone and Soft Tissue Tumors July-Dec 2018;4(2): 20-22.

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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.

References

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22. Cleven AH, Sannaa GA, Briaire-de Bruijn I, Ingram DR, van de Rijn M, Rubin BP et al. Loss of H3K27 tri-methylation is a diagnostic marker for malignant peripheral nerve sheath tumors and an indicator for an inferior survival. Mod Pathol 2016 J;29:582-90.
23. Pelmus M, Guillou L, Hostein I, Sierankowski G, Lussan C, Coindre J-M. Monophasic fibrous and poorly differentiated synovial sarcoma: immunohistochemical reassessment of 60 t(X;18)(SYT-SSX)- positive cases. Am J Surg Pathol 2002;26:1434–40.
24. Dei Tos AP, Calonje E, Sciot R, Pauwels P, Knight JC, Dal Cin P et al. Immunohistochemical demonstration of glycoprotein p30/32MIC2 (CD99) in synovial sarcoma. A potential cause of diagnostic confusion. App Immunhistochem 1995;3:168-73.

How to Cite this article: Kiruthiga KG, Anne Jennifer P, Rekha P, Sramana M, Reka.K, V.T.K.Titus, Selvamani B.. Biphasic synovial sarcoma – A ten year experience with molecular profile and clinical outcome. Journal of Bone and Soft Tissue Tumors July-Dec 2018;4(2): 14-19.

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Osteosarcoma of Extragnathic Skull Bones-clinicopathological Profile of Eight Cases

Vol 4 | Issue 2 | July-Dec 2018 | Page 11-13 | Mahfooz Basha Mohamed, Jayasree Kattoor, Kusumakumary Parukuttyamma, Geetha Narayanan, Anitha Mathews, Thara Somanathan.

Authors: Mahfooz Basha Mohamed [1], Jayasree Kattoor [2], Kusumakumary Parukuttyamma [3], Geetha Narayanan [4], Anitha Mathews [2], Thara Somanathan [2].

[1] Department of Laboratory Medicine, GKNM Hospital, Coimbatore 641006, Tamil Nadu, India.
[2] Department of Pathology, Regional Cancer Centre, Trivandrum 695011, Kerala, India.
[3] Department of Paediatric Oncology, Regional Cancer Centre, Trivandrum 695011, Kerala, India.
[4] Department of Medical Oncology, Regional Cancer Centre, Trivandrum 695011, Kerala.

Address of Correspondence
Dr. JayasreeKattoor,
Department of Pathology, Regional Cancer Centre, Trivandrum-695011, Kerala, India.
Email: jayasreeramdas@gmail.com

Abstract

Osteosarcoma is the most common primary malignant tumor of bone, usually arising from the metaphysis of the long bones around the knee joint. In 6-13% cases they are located in the head and neck region, of which maxilla and mandible are the most common sites. Osteosarcoma involving the extra-gnathic craniofacial bones account for less than 2% cases. We report eight such cases of osteosarcoma involving this unusual location in the last three years (2011 -2014) and present their clinicopathological profile. Seven patients were under 15 years of age and one patient was 37 years old. Out of the eight cases, four were males and four were females. The location of the tumor included occipital bone, parietal bone, external auditory canal, nasal bone and mastoid. Two patients presented as multicenteric disease with multiple lesions in the skull and elsewhere. Two patients succumbed to the disease while five patients are on follow up. One patient was lost to follow up.A complete en-bloc dissection of the tumor with free margins is a challenge for the operating surgeons. Radiologically they can simulate non-neoplastic lesions or benign tumors as well.These tumors pose a unique therapeutic challenge owing to their unusual location and require a multidisciplinary team approach for management of the patient.
Keywords: Extragnathic, skull, bone, osteosarcoma.

References

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How to Cite this article: Mohamed MB, Kattoor J, Parukuttyamma K, Narayanan G, Mathews A, Somanathan T. Osteosarcoma of Extragnathic Skull Bones-clinicopathological Profile of Eight Cases. Journal of Bone and Soft Tissue Tumors July-Dec 2018;4(2): 11-13.

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Spectrum of Bone and Soft Tissue Tumors in A Tertiary Cancer Institute in Eastern India

Vol 4 | Issue 2 | July-Dec 2018 | Page 7-10 | Ashwini Natekar, Gaurav Gupta, Subhasis Basu.

Authors: Ashwini Natekar [1], Gaurav Gupta [1], Subhasis Basu [1].

[1] Dept of Pathology, Chittaranjan national cancer institute, Kolkata, India

Address of Correspondence
Dr. Ashwini Natekar,
Dept of Pathology, Chittaranjan national cancer institute, Kolkata, India

Abstract

Introduction: Bone and soft tissue tumours are uncommon tumours that can affect any age group. Soft tissue tumors are said to be heterogeneous group of mesenchymal malignancies. Primary bone sarcomas are rare tumors, comprising approximately 0.2% of all cancers. Their true incidence is difficult to estimate because of their rarity.[1]The outcome of the disease depends on the age and time at diagnosis.
Material and Methods: A study was carried out in department of pathology in tertiary cancer institute in East India from December 2015 to September 2017. A total of 60 cases were included in the study. Clinical and radiological details of patients were noted along with gross specimen findings and microscopic examination of H &E stained slides. IHC was also carried out for confirmatory diagnosis.
Aims and Objecctives: 1- To study the histological spectrum of bone and soft tissue tumors in a tertiary cancer institute in Eastern India
2- To study gender distribution and site distributionof bone and soft tissue tumors
Results: Out of the total 60 cases studied 24 cases were of bone and cartilaginous tumours and 36 cases included were of soft tissue tumors. Benign tumors comprised of 08 cases (13.3%) and malignant tumors accounted to 52 cases (86.7%). Age of the patients ranged from 10 to 80 years. Male female ratio was 1.4:1.
Conclusion: Multimodal therapies of treatment is practiced for bone and soft tissue tumors. Early detection and treatment is essential for diagnosis of these malignant tumors. Molecular studies are most important in diagnosing, classifying and also prognosticating bone and soft tissue tumors.
Keywords: bone tumors, soft tissue tumors, histology.

References

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18. AF Mavrogenis,1 G Bianchi,2 NA Stavropoulos,1 PJ Papagelopoulos,1 and P Ruggieri2 Clinicopathological features, diagnosis and treatment of clear cell sarcoma/melanoma of soft parts Hippokratia. 2013 Oct-Dec; 17(4): 298–302.
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20. O. Hocar,1 A. Le Cesne,2 S. Berissi,1 P. Terrier,2 S. Bonvalot,2 D. Vanel,3 A. Auperin,4 C. Le Pechoux,2 B. Bui,5 J. M. Coindre,5 and C. Robert1Clear Cell Sarcoma (Malignant Melanoma) of Soft Parts: A Clinicopathologic Study of 52 Cases:Dermatology Research and Practice

How to Cite this article: Ashwini Natekar A, Gupta G, Basu S. Spectrum of Bone and Soft Tissue Tumors in A Tertiary Cancer Institute in Eastern India. Journal of Bone and Soft Tissue Tumors July – Dec 2018;4(2): 7-10.

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Guest Editorial – Asia Pacific Musculoskeletal Tumor Society Conference 2018

Vol 4 | Issue 2 | July-Dec 2018 | page: 2-3 | Ajay Puri.

Author: Ajay Puri [1].

[1] Orthopedic Oncology Services, Department of Surgical Oncology, Tata Memorial Hospital, Mumbai.

Address of Correspondence
Dr. Ajay Puri,
Department of Orthopaedic Oncology, Room No: 26, Tata Memorial Hospital, Borges Road, Parel, Mumbai – 400 012. India.
Email: docpuri@gmail.com

Asia Pacific Musculoskeletal Tumor Society Conference 2018

For advances in musculo skeletal oncology to occur we need a host of clinical specialties along with nursing, rehabilitation and support staff interacting on a continuous basis. Sarcomas are rare and each individual and institution can only benefit from having access to pooled information and experience. For sarcoma care to evolve, ideas to surface and multi institute or multi-disciplinary collaborations to develop in the fields of basic research, patient care, biomaterials and prosthesis, there is a need for a common platform where all of us involved in the treatment of sarcomas can interact. Our socio economic milieu in the Asia Pacific region is unique. We have a spectrum ranging from resource rich societies with extensive state supported health care to resource constrained nations where patients have inconsistent access to health care and funding for health care is limited. Solutions and protocols applicable to the Western world need not necessarily be the most suitable in our scenario. Our large numbers coupled by the sheer native ingenuity necessary to offer quality health care in a resource challenged population can help us develop solutions that can be adapted everywhere.

The Asia Pacific Musculoskeletal Tumor Society (APMSTS) was conceptualized during discussions at the 7th International Symposium of Limb Salvage Surgeons (ISOLS) meeting in 1993 and its first meeting was held in 1995 in Japan under the stewardship of Professor Yoshio Ogihara. Mirroring a similar initiative, the Indian Musculo Skeletal Oncology Society (IMSOS) was established in 2013 to “promote scientific, evidence based, comprehensive multidisciplinary management of bone and soft tissue sarcomas and encourage basic and clinical research”. This year Jaipur, India plays host to the 12th APMSTS meeting in October. This is a joint meeting co-hosted with IMSOS. The theme of the conference is “Education – Collaboration – Innovation”. This epitomizes our desire to share and disseminate knowledge, the spirit of collaboration necessary to find answers to common problems and the need to derive innovative solutions best suited to our socio economic milieu.


The conference logo depicts the feathers of the peacock in the Indian national colours adorning a group of people with interlinked arms who represent the collaboration and camaraderie between individuals and countries that form the Asia Pacific Musculoskeletal Society. The logo represents the beauty and spirituality of India while signifying the desire to showcase skills from the Asia Pacific region. The peacock, the national bird of India symbolizes many of the qualities that we as “healers” must inculcate. In Hinduism, the peacock is associated with Saraswati the goddess of wisdom and learning. In Buddhism, the peacock is a symbol of purity and the ‘eyes’ in the peacock’s tail represent a symbol of watchfulness. The figure of the peacock is painted in various Islamic religious buildings while in Christianity the peacock was known as the symbol of resurrection and renewal. Just as the peacock elegantly unfolds its vibrant colours during its ritual dance, so too this conference is an occasion for us to demonstrate the best from all participating delegates.

We have more than 300 delegates from 28 countries participating in this meeting. Deviating from the accepted norm of didactic lectures the meeting focusses more on interactive sessions that will promote healthy discussion. There are case based panel discussions and sessions where senior musculoskeletal oncologists share their diverse experiences so that all attendees can benefit from the collective experience of others in order to enable them to make better decisions when managing similar cases. While providing adequate sessions for free papers there are two “show piece” orations. The IMSOS oration “Lessons learned from the European-American Osteosarcoma Study EURAMOS1” will be delivered by Prof. Stefan Bielack while the inaugural APMSTS “Prof. Yoshio Ogihara Oration” will be delivered by Dr. Suresh Nathan. The conference is preceded by multidisciplinary workshops that encompass all specialties involved in sarcoma care, offering delegates an opportunity to interact with faculty in smaller groups.

This unique joint meeting also offers the ideal platform to unveil a brief monograph “IMSOS guidelines for musculoskeletal sarcomas”, IMSOS’s endeavour to standardise the management of musculoskeletal sarcomas in India.

All our efforts are ultimately focussed on improving health care for our patients. A meeting such as this would be incomplete unless we involved them too. The IMSOS sarcoma support initiative will simultaneously conduct a parallel session that helps patients share their experience and presents an occasion where we can felicitate support personnel who selflessly give their time to enable us to optimise overall patient care.

We are sure the ambience and hospitality of Jaipur combined with an exciting and informative scientific programme will make this joint APMSTS – IMSOS conference a memorable and enriching event for all delegates.
It is our pleasure and privilege to welcome all attendees to Jaipur to partake of the experience that is “Incredible India”!

Prof. Ajay Puri
President – Indian Musculo Skeletal Oncology Society
President – Asia Pacific Musculoskeletal Tumor Society

How to Cite this article: Puri A. Asia Pacific Musculoskeletal Tumor Society 2018 Issue. Journal of Bone and Soft Tissue Tumors July-Dec 2018; 4(2):2-3.

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