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Osteosarcoma – A Clandestine Enigma

January 10, 2016

Vol 2 | Issue 1 | Jan – Apr 2016 | page:6-7 | Ashish Gulia. Author: Ashish Gulia[1] [1] Orthopaedic Oncology, Tata Memorial Hospital, Mumbai, India. Address of Correspondence Dr. Ashish Gulia MS (Ortho), Mch – Surgical Oncology Fellowship – Musculoskeletal Oncology (TMH – HBNI) Asst. Professor – Orthopaedic Oncology, Tata Memorial Hospital, Mumbai, India Osteosarcoma […]

Extraskeletal Myxoid Chondrosarcoma- Rare ‘Non-chondroid’ soft tissue Sarcoma!!!

January 10, 2016

Volume 2 | Issue 1 | Jan-Apr 2016 | Page 36-38 |Shital Biradar, Sujit Joshi, Yogesh Panchwagh, Vikram Ghanekar, Pradeep Kothadiya. Authers: Shital Biradar[1], Sujit Joshi[1], Yogesh Panchwagh[2], Vikram Ghanekar[3], Pradeep Kothadiya[4]. [1]Dept. of Pathology, Deenanath Mangeshkar hospital, Pune. [2]Histopathologist, Deenanath Mangeshkar hospital, Pune. [3]Orthopedic Onco-surgeon, Deenanath Mangeshkar hospital, Pune. [4]Surgical oncologist, S.G.M. Hospital; Chiplun. […]

Osteosarcoma – A Clandestine Enigma

January 10, 2016

Extraskeletal Myxoid Chondrosarcoma- Rare ‘Non-chondroid’ soft tissue Sarcoma!!!

January 10, 2016

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Osteosarcoma – A Clandestine Enigma

January 10, 2016

Vol 2 | Issue 1 | Jan – Apr 2016 | page:6-7 | Ashish Gulia.

Author: Ashish Gulia[1]

[1] Orthopaedic Oncology, Tata Memorial Hospital, Mumbai, India.

Address of Correspondence
Dr. Ashish Gulia MS (Ortho), Mch – Surgical Oncology
Fellowship – Musculoskeletal Oncology (TMH – HBNI)
Asst. Professor – Orthopaedic Oncology, Tata Memorial Hospital, Mumbai, India

Osteosarcoma – A Clandestine Enigma

Bone tumors form a small part of all human cancers. As per the SEER data about 2570 new cases of bone sarcomas were diagnosed in the United States in the year 2005. Osteosarcoma, earlier called as “Osteogenic Sarcoma” is the most common primary bone tumor in humans, which has a predilection for metaphysis of long bones in children, adolescents, and young adults and most commonly involves the bones around the knee joint in about 65% of cases. The earliest published literature takes us to 1879, where in his publication, Gross advocated early amputation as the only treatment modality for extremity osteosarcoma with dismal survival outcomes. Since then the overall outcome of osteosarcoma has seen a sea change. This journey of evolution of treatment of osteosarcoma has been a roller coaster ride with its ups and downs. The world has seen remarkable survival improvements with some rapid strides in 1970s and 1980s. Introduction of multi-agent chemotherapy improved the 5 year survival from a dismal 20% to almost 70%. This fast paced growth reached a stagnant phase with no further improvements in the survival in almost last three decades. Though multiple agents have been tested in both phase II and phase III randomized controlled trials, none has been significant enough to be incorporated in clinical practice. Similar to the oncological outcomes, functional outcomes have also seen a dramatic improvement over last half a century. Limb salvage has become a norm in today’s orthopedic oncology practice, which wasn’t so in 1970s. Advent of neo-adjuvant chemotherapy, refinements in surgical skills, availability of durable metallic endo-prosthesis have led to a “limb salvage revolution” where about 85% to 90% of extremity osteosarcoma patients use their own extremity at the end of the treatment. The exponential growth in function preservation still continues with more technology driven innovations and solutions making the commonly encountered implant related complications like aseptic loosening and frequent breakage, a thing of the past. The current standards of care warrant a multi-disciplinary approach in the management of osteosarcoma. The approach is not only required in treatment phase involving a multi-agent neo adjuvant chemotherapy followed by optimal surgical resection & reconstruction and adjuvant chemotherapy, but also in evaluation, diagnosis and staging process. A seamless integration between musculoskeletal surgeon, musculoskeletal radiologist and sarcoma pathologist can achieve higher levels of accuracy in diagnosis in order to initiate the optimum line of treatment within an ideal time frame. The presence of metastatic disease at presentation is one of the most significant negative prognostic factors. Western data have shown that about 15% to 20% of patients will have clinically detectable metastases at presentation with lung being the most common site of metastasis in about 85% of cases followed by bone as the second most common site. These figures may be higher in developing countries as patients typically present with large volume disease. Lack of awareness, belief in alternative medicine and poor socio economic status are some of the factors contributing to higher percentages in these countries. Delay in diagnosis due to lack of suspicion and inappropriate initial evaluation as well as management has also led to dismal outcomes. The present symposium on Osteosarcoma tries to address the above issues and provide evidence based robust data, which will help the clinicians to understand the principles for evaluation and management of extremity osteosarcoma. The importance of understanding the presenting symptomatology and clinical evaluation is well scripted in the first article [1]. This article also stresses the importance of multi disciplinary strategy to diagnose a suspected bone lesion correctly. It discusses in depth the role of sequential radiological and histopathological evaluation of a suspected case of osteosarcoma. Staging of osteosarcoma is also discussed, which eventually helps clinicians to plan the treatment and estimate the prognosis. Radiological evaluation, whether it is with radiographs or with high end cross sectional imaging, has been the cornerstone for diagnosis and the local staging of the disease. Osteosarcoma exhibits various radiological and histological forms, which have deep implications on their treatment. These varied radiological presentations are discussed in the second article, which gives a tabulated comparison of various characteristics and their differentials [2]. The modern era is dominated by technology driven tools and this surge is quite evident in evaluation of bone tumors too. Emergence of PET scan as a “one stop shop” for the evaluation of bone tumors has created some whirlpools, leading to unending debates in recent era. Though more data is being collected to prove its worth in osteosarcoma, it is now being used to replace invasive investigations in other tumors like Ewing sarcoma and chondrosarcoma. The third article discusses the use of PET scan as a single modality to stage as well as to assess the chemo response evaluation in osteosarcoma [3]. The recent advances of this bio-imaging tools and the probable futuristic avenues are addressed in the third article. Complete surgical resection has been the single most important criteria to achieve adequate local control. Local relapses are associated with very poor overall survivals. The surgeons need to work to achieve a fine balance between complete tumor resection and retaining function. Over the years this has been addressed by “concept of margins” which was first popularized by the godfather of musculoskeletal oncology, Dr. W.F. Enneking. The concept was further revisited and modified by Kawaguchi, who gave the concept of “barrier effects” and challenged the traditionally propagated concept of “quantitative margins” and replaced it with a new concept of “qualitative margins”. The fourth article in the symposium address the similar issues regarding the adequacy of margins in the resection of osteosarcoma [4]. The article also explains the relationship of local failures with respect to resection margins and tumor necrosis. As discussed earlier multi-agent chemotherapy forms an integral part of management of osteosarcoma. The next article details the evolution of various chemotherapy protocols and current standards of chemotherapy for osteosarcoma [5]. Osteosarcoma is considered as a radio-resistant tumor, thus radiotherapy had a limited role in the management of osteosarcoma. Similar to the other specialties of medicine, radiation oncology has seen major changes in understanding the mechanism of radio tumor kill and also in the development of the delivery system. The advent of high-end technique like carbon ion and proton beam radiotherapy with their high accuracy, ability to give very high focused dosage and reduced side effects have made radiotherapy a new tool in the armamentarium for local control in osteosarcoma. Though these techniques are more useful in non-resectable lesions of axial skeleton, these are becoming increasingly popular in margin positive cases to avoid amputations. The latest updates regarding the use of this modality are explained in the last article of the symposium [6]. This symposium on osteosarcoma has been divided in to two sets, this first set encompasses articles related to evaluation and overall management of extremity osteosarcoma. Next issue will contain the second half of the symposium which will have articles on surgical management and advances in the management of osteosarcoma.

References

1. Shah M, Anchan C. Evaluation of Osteogenic Sarcoma. Journal of Bone and Soft Tissue Tumors Jan-Apr 2016;2(1):8-12.
2. .Janu A, Jain N, Juvekar S, Gulia A. Radiological Review of Extremity Osteosarcoma. Journal of Bone and Soft Tissue Tumors Jan-Apr
2016;2(1):13-18.
3. Purandare NC, Rangarajan V. Emerging role of PET/CT in osteosarcoma. Journal of Bone and Soft Tissue Tumors Jan-Apr 2016;2(1):19-21.
4. Cloake T, Jeys L.How important are surgical margins in Osteosarcoma? . Journal of Bone and Soft Tissue Tumors Jan-Apr 2016;2(1):22-26
5. Jain S, Kapoor G. Chemotherapy in Osteosarcoma: Current Strategies. Journal of Bone and Soft Tissue Tumors Jan-Apr 2016;2(1):27-32.
6. Kakoti S, Khanna N, Laskar S. The Current Role of Radiation Therapy for Osteogenic Sarcoma. Journal of Bone and Soft Tissue Tumors Jan-Apr
2016;2(1):33-35.

How to Cite this article: Gulia A. Osteosarcoma – A Clandestine Enigma. Journal of Bone and Soft Tissue Tumors Jan-Apr 2016;2(1): 6-7.

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Extraskeletal Myxoid Chondrosarcoma- Rare ‘Non-chondroid’ soft tissue Sarcoma!!!

January 10, 2016

Volume 2 | Issue 1 | Jan-Apr 2016 | Page 36-38 |Shital Biradar, Sujit Joshi, Yogesh Panchwagh, Vikram Ghanekar, Pradeep Kothadiya.

Authers: Shital Biradar[1], Sujit Joshi[1], Yogesh Panchwagh[2], Vikram Ghanekar[3], Pradeep Kothadiya[4].

[1]Dept. of Pathology, Deenanath Mangeshkar hospital, Pune.
[2]Histopathologist, Deenanath Mangeshkar hospital, Pune.
[3]Orthopedic Onco-surgeon, Deenanath Mangeshkar hospital, Pune.
[4]Surgical oncologist, S.G.M. Hospital; Chiplun.
[5]Orthopedic Surgeon, Kothadiya Hospital; Solapur.

Address of Correspondence
Dr. Sujit Joshi
Flat No: 2, Lunawat Reality, Opp. Vanaz Company, Paud road, Kothrud, Pune-411038.
Email ID: sujitjoshi30@gmail.com

Abstract

Extraskeletal myxoid chondrosarcoma (ESMC) is an uncommon but distinct entity with clearly different clinicopathological, immunohistochemical and cytogenetic features from those of conventional skeletal chondrosarcoma. Because of its better prognosis as compared to conventional skeletal chondrosarcoma, an accurate diagnosis is essential. We present 2 cases of this tumor with different clinical presentations.
1. 40 year old house wife presenting with a 9x8cm size mass around lower end of femur. On imaging, it was a soft tissue mass abutting the femoral surface with minimal bone invasion.
2. 50 year old lady presenting with a huge fungating soft tissue mass over left lower leg and foot associated with similar cutaneous nodules over right arm and left thigh. The left leg mass had caused destruction of entire lower end of fibula.
Histopatholgical evaluation of both cases showed features of Extraskeletal Myxoid Chondrosarcoma (ESMC). Characteristic histopathological features include a malignant soft tissue neoplasm with lobulated growth pattern, abundant myxoid matrix and fairly bland looking tumor cells. There is no convincing evidence of cartilagenous differentiation or chondroid matrix production. Immunohistochemistry has a limited role.
ESMC is a tumor with long survival but a prolonged follow up is necessary in view of high local recurrence, high metastatic rates and high disease related mortality. The diagnosis of this tumor largely depends upon knowledge of this entity and its characteristic histopathological features.
Key words: Extra-skeletal myxoid chondrosarcoma; ESMC.

Introduction

Extraskeletal myxoid chondrosaroma (ESMC) is a rare malignant soft tissue sarcoma described as a distinct clinico-pathologic entity by Enzinger and Shiraki in 1972 [1]. WHO categorized this tumor as a tumor of uncertain differentiation since there is paucity of convincing evidence of cartilagenous differentiation. It is a rare tumor, accounting for less than 3% of soft tissue sarcomas [2,3]. The tumor usually develops in deep parts of the proximal extremities and in middle-aged adults [4]. More than two-thirds of the tumors occur in the proximal extremities and limb girdles, especially the thigh and popliteal fossa. Here, we are presenting the detailed clinico-pathological findings of two such cases, which were diagnosed in our institute.

Case Report

Case 1: 40 year old housewife presented with lower limb swelling and pain just above the knee joint which started since 5 months (Fig 1). Plain radiographs reveal a soft tissue mass abutting the femoral surface with minimum bony involvement in supracondylar region of femur suggestive of a soft tissue neoplasm (Fig. 2). MRI revealed a large extra-osseous soft tissue lesion with minimal intra-osseous extension suggestive of a juxtacortical neoplasm or soft tissue sarcoma (Fig. 3). Open biopsy was done elsewhere and reports were reviewed at authors institute. It showed microscopic features of ESMC. Considering the interosseous involvement and safe oncological margins, a wide resection was planned. The patient underwent limb salvage surgery in form of wide local excision of distal femur along with the mass and reconstruction with a megaprosthesis. Pathological Findings: On gross examination of the wide local excision specimen of distal femur, there was an extra-osseous mass at the lower end of femur on the postero-lateral aspect measuring 9×8 cm in size. It was a soft to firm, ovoid, lobulated mass which on cut section, was gelatinous, mucoid and gray-white. There was no evidence of necrosis or haemorrhage noted (Fig 4). Microscopically, it showed a characteristic multi-nodular pattern. Tumor cells were small round with hyperchromatic nuclei and a narrow rim of cytoplasm (Fig 5a). Cells were arranged in cords and strands separated by abundant myxoid material (Fig 5b,c). No cartilaginous matrix production was seen in the stroma. On Immuno-histochemestry (IHC) the cells showed strong positivity for Vimentin and focal positivity for S-100 protein. These cells were negative for Cytokeratin, EMA, Synaptophysin and Chromogranin. Based on morphological and IHC findings, a diagnosis of Extra-skeletal myxoid chondrosarcoma (ESMC) was reached.

Case 2: 50 year old lady presented with a huge fungating soft tissue mass over left foot associated with similar cutaneous nodules over right arm and left thigh. The foot lesion was progressively increasing over a year but not associated with pain. (Figure 6a, 6b)
X-ray of left foot and lower limb revealed a large soft tissue mass destroying the metatarsals , devoid of any matrix and periosteal reaction (Fig. 7a). Similar lesion was seen destroying the ipsilateral distal fibula (Fig. 7b). Biopsies from the foot mass and the arm nodule revealed histopathological and IHC findings consistent with Extraskeletal Myxoid Chondrosarcoma (ESMC). This patient defaulted for further treatment and follow up.

Discussion:
Extraskeletal myxoid chondrosarcoma (ESMC) is a rare, morphologically distinct soft tissue sarcoma with characteristic nodular architecture & abundant myxoid matrix. In 1972, Enzinger and Shiraki were the first ones who coined ESMC as a distinct entity [1]. In spite of it’s name, Extraskeletal myxoid chondrosarcoma (ESMC) is considered as a “Tumor of uncertain differentiation” because there is no definite evidence of cartilage matrix production in the tumor. Histogenesis of ESMC is still subject of controversy. Incidence of this tumor is only 2.3% of all soft tissue sarcomas as reported by Tsuneyosi et al [2]. Mainly the adult age group (35 years and above) is affected by this tumor, with equal sex predilection [3]. Most common sites are deep soft tissues of the proximal extremities and limb girdles, especially the musculature [4]. However, few uncommon sites are also been described like mediastinum, retroperitoneum, abdomen and the digits [5-7]. In both of our cases, imaging showed that it was a lobulated soft tissue mass without bony periosteal reaction or any radiologically evident matrix production. Histopathologically, both cases showed presence of uniform eosinophilic cells arranged in cords & deposited in an abundant myxoid stroma. There was no evidence of cartilage/osteoid matrix deposition.
The most important clue to the diagnosis of this rare entity is the typical arrangement of cells in cords and columns with a very prominent myxoid background [8]. ESMC appears to exhibit a high tendency of local recurrence & distant metastases, predominantly to the lungs, sometimes years after the initial diagnosis [9]. ESMC should be considered an intermediate grade tumor rather than a low-grade malignant neoplasm as the estimated 5, 10 and 15-year survival rates described by Meis-Kindblom et al were 90%, 70% & 60% respectively [4]. Wide local excision of the tumor is the treatment of choice. If a wide margin can not be obtained, a high rate of local recurrence is observed with poor response to chemotherapy & radiotherapy. Therefore, surgery with appropriate adequate margins continues to be the treatment of choice, for primary as well as recurrent or metastatic tumors.
On follow up part, our first patient is disease free at 6 years from date of surgery with excellent function in the operated limb (MSTS score 97%). The second patient defaulted for further treatment and was lost to follow up. Some adverse pathological prognostic factors reported in literature include Tumor size ≥ 10 cm, high cellularity, anaplasia or rhabdoid features, mitotic activity more than two per 10 high-power fields, and Ki-67 proliferative index of ≥ 10%. These indicate more aggressive behavior, requiring a closer follow-up of the patient [10]. In summary, ESMC is an uncommon but distinct soft tissue sarcoma, clearly different from conventional skeletal chondrosarcoma. Knowledge of this entity and accurate diagnosis is essential because of the difference in its behaviour and prognosis.

References

1. Enzinger FM, Shiraki M. Extraskeletal myxoid chondrosarcoma. An analysis of 34 cases.Hum Pathol 3: 421-35,1972.
2. Tsuneyoshi M, Enjoji M, Iwasaki H and Shirahama N: Extraskeletal myxoid chondrosarcoma: a clinicopathologic and electron microscopic study. Acta Pathol Jpn 31: 201-208, 1981.
3. Antonescu CR, Argani P, Erlandson RA, et al: Skeketal and extraskeletal myxoid chondrosarcoma: a comparative clinicopathologic, ultrastructural, and molecular study. Cancer 83:1504-1521, 1998.
4. Meis-Kindblom JM, Bergh P, Gunterberg B, et al: Extraskeletal myxoid chondrosarcoma: a reappraisal of its morphologic spectrum and prognostic factors based on 117 cases. Am J Surg Pathol 23: 636-650, 1999.
5. Oliveira AM, Sebo TJ, McGrory JE, et al: Extraskeletal myxoid chondrosarcoma; A clinocopathologic, immunohistochemical, and ploidy analysis of 23 cases. Mod Pathol 13: 900-908, 2000.
6. Patel SR, Burgess MA, Papadopculos NE, Linke KA and Benjamin RS: Extraskeletal myxoid chondrosarcoma: long-term experience with chemotherapy. Am J Clin Oncol 18: 161-163,
1995.
7. Okamoto S, Hara K, Sumita S, et al: Extraskeletal myxoid chondrosarcoma arising in the finger. Skeletal Radiol 31: 296-300, 2002.
8. Jakowski JD, Wakely PE Jr. Cytopathology of extra-skeletal myxoid chondrosarcoma: Report of 8 cases. Cancer 2001; 111:298-305
9. Weiss SW and Goldblum JR: Extraskeletal myxoid chondrosarcoma. In: Soft Tissue Tumors 4th Edition. Mosby, St. Louis,pp1368-1379, 2001.
10. Oliveria Am, Sebo TJ, McGrory JE, Gaffey TA, Rock MG, Nascimento AG. Extraskeletal myxoid chondrosarcoma: A clinicaqopthologic, immunohistochemical & ploidy analysis of 23 cases. Mod Pathol 2000; 13:900-8.

How to Cite this article:Biradar S, Joshi S, Panchwagh Y, Ghanekar V, Kothadiya P. Extraskeletal Myxoid Chondrosarcoma- Rare ‘Non-chondroid’ soft tissue Sarcoma!!!. Journal of Bone and Soft Tissue Tumors Jan-Apr 2016;2(1):36-38 .

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Osteosarcoma – A Clandestine Enigma

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