OncoMedia Journal of Bone and Soft Tissue Tumors (JBST) May-August 2015

Vol 1 | Issue 1 | May – August 2015 | page:51-52 | Dr Ashish Gulia[1], Dr Ashok Shyam[2,3].

Author: Dr Ashish Gulia [1], Dr Ashok Shyam [2,3].

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

Dr Ashok Shyam MS (Ortho)
[2]Indian Orthopaedic Research Group, Thane, India.
[3] Sancheti Institute for Orthopaedics &Rehabilitation, Pune, India.

Indian Musculo Skeletal Oncology Society (IMSOS)

Musculoskeletal oncology is a relatively new specialty, both as far as orthopaedics and oncology goes. 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. This would also help foster training and education opportunities, promote dissemination of knowledge and aid in the development of treatment guidelines suitable for our socio cultural environment. As the field of musculoskeletal oncology continues to develop globally and in India, it is time for us to reflect on what is required for it to grow further so that we are able to offer the best care to the maximum number of patients.
The Indian Musculo Skeletal Oncology Society (IMSOS) is a step in this direction. It aims to “promote scientific, evidence based, comprehensive multidisciplinary management of bone and soft tissue sarcomas and encourage basic and clinical research.” In the words of Henry Ford “Coming together is a beginning; keeping together is progress; working together is success”.
The Indian Musculoskeletal Oncology Society 1st Annual Conference was organised on 13th and 14th March, 2015 at the Tata Memorial Hospital, Mumbai.
The theme of the conference was “Cure, control or comfort – In tumors teamwork triumphs!” reflects the ethos of IMSOS of bringing together all specialties interested in sarcoma care to interact on a continuous basis and help further advances in musculoskeletal oncology. The second meeting is planned in Cochin in 2016. Visit www.imsos.org for more information.

Bone marrow as a metastatic niche for disseminated tumor cells from solid tumors

Tissue specificity of tumor cells to metastasize, for example predilection of lung carcinoma to spread to bone, is still poorly understood. It is believe that the tumor cells with seed into tissues that act as good soil form them to grow. In this respect bone marrow is said to be the metastatic niche for seeding and growth of variety of tumors. The tumor cells mimic the homeopoetic stem cells and capture the niche for themselves through series of complex steps involving cytokines, adhesion molecules and physical factors. The detailed mechnism still eludesus but we do grasp the importance of this phenomenon. This colonisation may play important role in cases that relapse after chemotherapy. In these cases the tumor cells may find safe haven in the bone marrow niche and can emerge later to cause further metastasis and disease spread. Understanding of these mechanism will help in developing effective chemotherapeutic solutions and may allow to restrict the disease for spreading too. Recent article published in BoneKey1 elaborates on the function of metastatic niche and provides insight into new developments to tackle this. But we are still far away from any clinical implication of the theory.
1. Shiozawa Y, Eber MR, Berry JE, Taichman RS. Bone marrow as a metastatic niche
For disseminated tumor cells from solid tumors. Bonekey Rep. 2015 May 20;4:689Osteoclasts cause muscle weakness and bone pain in bone tumorsOsteoclasts cause muscle weakness and bone pain in bone tumors.

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Osteoclasts cause muscle weakness and bone pain in bone tumors

Understanding the basic mechanisms by which tumors cause certain systemic symptoms will help in understanding and planning therapeutic strategies. Tumor cells stimulate osteoclastic activity which lead to outpour of excess of bone derived growth factor. The bone derived growth factors have direct effect on the muscles and cause muscle weakness as well as muscle wasting. It is believed that in this muscle-bone synergy it’s the muscle that is a more powerful secretary gland and has strong influence on bone homeostasis. However in cases with bone tumor this relationship is reversed and also distorted leading to muscle wasting. Most important factor is TGF-beta family of ‘osteokines’. These may cause reduction is both muscle mass as well as muscle function. The weak bones add to the impaired function. The same hyperactive osteoblasts create an acidic environment in the bone which is directly related to severity of bone pains. Two good article clear a lot of confusion and provide fresh insights into the subject[1,2].

1. Waning DL, Guise TA. Cancer-associated muscle weakness: What’s bone got to do
with it? Bonekey Rep. 2015 May 20;4:691.
2.Nagae M, Hiraga T, Yoneda T. Acidic microenvironment created by osteoclasts
Causes bone pain associated with tumor colonization. J Bone Miner Metab. 2007;25(2):99-104.

Hair Bucket Challenge’ helping Sherwood boy with Ewing’s Sarcoma

Social impact of bone tumors are not unknown but this is unique. We all know about the ice bucket challenge, but an hair bucket challenge is unheard of. This was created for fourth-grader Tony Budesilich, who was recently diagnosed with Ewing sarcoma, a rare bone cancer in his leg (fibula which was surgically excised. The boy underwent seven rounds of chemotherapy and started losing hair. His friends noticed this change specifically Aidan Cook (12 yrs) who shaved his head and thus started a challenge the other boys to shave theirhead. This became an internet sensation and lot of kids [not only friends of Tony] from the locality participated and we could see a lot of shaved head in Sherwoods.
Tony has been given a good prognosis but has to continue the complete course of chemotherapy, but he definitely feels great about how his friends and family have supported him in a very difficult phase of his life

Read more: http://www.kptv.com/story/29109392/hair-bucket-challenge-helping-sherwood-boy-with-rare-bone-cancer#ixzz3d8e4tHuP.

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Join the OncoMedia Team and keep the interesting news coming through. We invite trainees of all faculties involved in the care of bone and soft tissue tumors to become a part of this active and dynamic team. They will be required to search the web and find interesting news and facts (which we will otherwise miss) and send it to the editorial board. A short original write-up will be necessary for the same. If accepted your news article will be published with your photograph and affiliation.  To be a part of OncoMedia please write an email to us at editor.jbst@gmail.com

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Ewing Sarcoma: Focus on Surgical Management

Vol 1 | Issue 1 | May – August 2015 | page:23-28 | Yogesh Panchwagh[1*].

Author: Yogesh Panchwagh[1*].

[1]Orthopaedic Oncology Clinic, 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

Abstract

Ewing sarcoma is one of the common primary bone sarcomas affecting patients mostly in the second decade. Appropriate clinical examination, investigations, staging, biopsy and multi-modal treatment are essential for good outcome. Neo-adjuvant and adjuvant chemotherapy have shown definite benefits in local and systemic control and in improving survival. Though historically, emphasis of treatment was on radiation, non metastatic Ewing sarcoma is shown to have better outcome with surgical excision as compared to definitive radiotherapy. Limb salvage surgery is currently the norm given the excellent functional outcomes. Various reconstruction options are available depending upon the age, site and size of the lesion. Appropriate follow up is essential to pick up local and systemic failures early. Individualized approach may be required for patients who are metastatic at presentation.
Keywords: Ewing sarcoma, Surgery, Limb salvage, reconstruction.

Introduction

Ewing Sarcoma (ES) is a highly aggressive malignant tumor affecting mostly the immature skeleton, more commonly in the second decade of life. ES is named after Dr. James Ewing, a pathologist. Its aetiopathogenesis has evolved from “Endothelioma of bone” to a unique malignant tumor of bone with well described translocation t(11;22)(q24;q12) as a possible causative factor [1]. The classical pathology of small round blue cells makes it a part of the Round cell family of tumors, the other members of which are rhabdomyosarcoma, synovial sarcoma, non-Hodgkin’s lymphoma, retinoblastoma, neuroblastoma, hepatoblastoma, and nephroblastoma or Wilms’ tumor [2].

Clinical presentation
A high index of suspicion is required to diagnose a primary bony sarcoma like ES at a very early stage. The patients, typically in their first two decades of life, usually have a history of 2-6 months duration, of a painful, progressively increasing swelling in the affected area. Most of the patients give a concomitant history of trauma, which is coincidental. Some of the patients may have a history of fever [3,4].
Clinical examination reveals a tender diffuse swelling in the affected area. The range of motion of the adjoining joint may be terminally restricted. The palpation may reveal local warmth. Though the most commonly affected site is the diaphysis in the bone, ES is known to affect the metaphyseal region as well [3]. ES may affect any bone in the body (Fig 1 a-e). Periosteal ES located on the surface of bone and soft tissue ES, though rare, are well-defined clinical entities [3].
‘The clinical and radiological features in an ES of bone may be akin to osteomyelitis or an eosinophilic granuloma and these differentials have to be borne in mind and ruled out by subsequent investigations.

Figure 1
Work up:
The work up includes plain radiographs of the affected bone including the nearby joint, M.R.I. scan of the involved bone, and either a PET CT [7,8,9] or a CT Chest with Technetium Bone scan and a bone marrow aspiration biopsy [3]. The x ray ( Figure 1, a-e) usually shows a permeative, lytic lesion with lamellated periosteal reaction. In locally advance cases, an extra osseous soft tissue component is common [1,3,5]. A diaphyseal lesion may exhibit a characteristic “Onion peel” periosteal reaction. In some cases, “hair-on-end” or “sun-ray spicule” type of periosteal reaction may also be seen.
The laboratory investigations may reveal leukocytosis with elevated E.S.R. and C.R.P [3]. Serum levels of Lactate Dehydrogenase (S. LDH) are usually elevated and serve as a marker of disease activity and response to treatment [6].
These clinico-radiological and laboratory parameters are akin to osteomyelitis and it requires a trained eye with high index of suspicion to pick the neoplastic nature early in order to avoid mistreating these patients. MRI scan (Fig 2 ) has emerged as one of the most important radiological investigation amongst the others, in the work-up of primary bone sarcomas. It helps immensely in delineating the marrow involvement, revealing skip lesions if any, understanding the extent of soft tissue component and its relationship with the neuro vascular bundle, joint involvement and to decide the ideal site for biopsy. M.R.I. can also be used to assess response to neo-adjuvant chemotherapy [3,5].

Staging
Staging in a case of ES is of paramount importance because of its bearing on the overall prognosis and treatment decisions [1,3]. The conventional staging investigations included a C.T. scan of the chest, a three phase technetium mendronate bone scan and a bone marrow aspiration biopsy [3,10]. However, with the advent of P.E.T. C.T., the bone marrow aspiration biopsy is being found unnecessary [8,9].

Biopsy
The clinico-radiological suspicion of Ewing sarcoma has to be corroborated by a biopsy and pathological examination before further treatment is commenced. The biopsy of such a lesion is to be done preferably by the orthopaedic oncologist who will be treating the case, at a multi disciplinary cancer centre [3, 11, 13, 14, 15, 16, 17]. Most of the lesions are accurately diagnosed by a needle biopsy. Under the microscope, the tumor is arranged in sheets, nests or clusters of small round blue cells invading the native bone [1]. (Fig 3 a,b). The cells show dense blue chromatin with scanty cytoplasm and the contained glycogen is evident by the P.A.S. (periodic acid-Schiff) stain positivity. Immunohistochemical markers as CD 99 (a mic-2 gene product) and Fli-1 are diagnostic of Ewing sarcoma and are used as confirmatory tools [1,3].

Figure 2 Figure 3

Treatment
The treatment of ES is handled by a multi disciplinary team comprising of the orthopaedic oncologist, Medical oncologist, Radiation Oncologist, Pathologist and Radiologist [11, 12, 13 , 16, 17, 18]. The patient and the family need to be informed about the clinical results and the expected prognosis and have to participate in the decision making process. Flowcharts of both diagnostic work up and management protocol are provided in figures 5 and 6. The prognosis depends upon the metastatic status of the patient, with the non-metastatic patients having a better outcome [1,3].
The actual management of non-metastatic ES requires neo-adjuvant chemotherapy, followed by surgical resection (if feasible and indicated) followed by post op radiotherapy if necessary (OR definitive local radiotherapy) and then adjuvant (post operative) chemotherapy [1,3] .
In general, the local treatment outcome of an extremity ES is better with surgical wide resection than compared to definitive local radiotherapy alone [18]. In an axially located ES as in pelvis and spine, the decision regarding excision will have to be weighed against the morbidity of the surgery [19]. In a non-metastatic axially located ES, surgery or combined surgery and radiotherapy appears to have an edge over only radiotherapy; the latter being used only in unresectable tumors [20, 21, 22, 23].
The neo adjuvant chemotherapy helps in multiple ways. It is useful in downstaging the local disease, reducing the vascularity, controlling the micro-metastases, sterilizing the satellite lesions in the surrounding zone of hyperemia, helping formation of a thicker capsule, reducing the local edema, healing of pathological fractures and prognosticating outcome of the treatment based on the analysis of percentage necrosis in the tumor. All of these help in making the surgical excision easier and reduce the local recurrence rates [24,25,26,27,28,29,30,31].
The decision regarding limb salvage in a non-metastatic case of Ewings sarcoma is based upon the local extent of the disease. The status of the neuro-vascular bundle and amount of muscles involved by the soft tissue component, determine feasibility of a limb salvage surgery. The only absolute contra indication to a limb salvage surgery would be encasement of a major motor nerve in the extremity and inadequate muscles left after wide excision of the lesion, which would result in a non-functional extremity.
In a case that there are metastases at diagnosis, the decision regarding the approach is based on the number and type of metastases. In a widely metastatic case, only palliative treatment is offered. If there are few pulmonary metastases amenable to excision or are of doubtful significance, the patient is given neo adjuvant chemotherapy and re-staged. The decision regarding treatment is then based on the response to the chemotherapy. If there is progression despite the neo-adjuvant chemotherapy, palliative protocol is followed. If the metastatic lesions have responded to the chemotherapy then the local treatment decision can be taken accordingly with curative intent [25, 28].
The local control rates and the overall survival rates for patients of primary bone sarcomas treated with limb salvage and for those treated by amputation are comparable, with limb salvage surgery carrying better functional outcome [26,28,30,34]. In developing countries, it is worthwhile to offer limb salvage to patients who have a better prognosis, in whom the function of the salvaged extremity is going to be acceptable and for those who are willing to complete the necessary treatment and understand the complications involved.
The exact modality of reconstruction after limb salvage is decided by the site of disease, the extent, the patients age [11,34] and expectations and in the developing world, by the socio-economic status of the patient (Fig 4). For periarticular ES, reconstruction can be done by using megaprosthesis [34] or allo-prosthesis composite. This restores the function in the operated extremity fast, shortens the rehabilitation time post operatively, enables early resumption of adjuvant treatment modalities, is a durable option with acceptable complication rate. Arthrodesis can be an alternative to megaprosthetic reconstruction. In cases with diaphyseal involvement, joint sparing inter-calary resections can be done and the defect reconstructed using allograft – live fibula composite or only live vascularised fibula or extra corporeal radiotherapy and reimplantation [35,41]. Rotationplasty is a viable alternative for very young children [36] and in cases of failed limb salvage surgery [37].
The post operative margins of the resected specimen and the percentage necrosis after chemotherapy decide the need for post operative radiotherapy. In cases where the margins are inadequate or the tumor is viable, radiation is used post operatively in order to achieve better control rates [3,21-27,29]. The adjuvant chemo continues in the post operative period. [3, 4, 22, 23, 24, 25, 26, 27, 30, 31, 34].
Patients treated thus need to undergo the prescribed rehabilitation program in order to attain the maximum functional outcome [38]. Functional outcomes in these patients are measured by the Musculo Skeletal Tumor society scoring system (MSTS) or the Toronto extremity salvage score (TESS) [39, 40]. These scores basically reflect the ability of the patient to carry out activities of daily living.

Figure 4

Follow up
The patients are advised to follow up every 3 monthly in the first two years, every six monthly for next three years and annually thereafter. At every visit, radiographs and appropriate staging investigations follow clinical examination [3,25]. Fuchs et al have reported long term complications in 59% percent of patients treated for ES over a average follow up of 25 years [46]. These complications comprised of metastases, local recurrence, secondary malignancies, pathologic fractures, and radiation-associated and chemotherapy-associated morbidities. Hence it is recommended to follow all these patients over a longer period.

Fig 5            Fig 6

Results
In various studies, the overall survival (at 3 or 5 year follow up) for non metastatic ES has been reported to be between 43.5% to 80% [1,23,42-48]. The local recurrence rates are reported to be around 10% to 12.5% [44,48]. In long term follow up of an average of 18 years, Bacci et al have reported overall survival at 5, 10, 15 and 20 years as 57.2%, 49.3%, 44.9% and 38.4% respectively [45]. The poor prognostic indicators in a case of ES are presence of metastases (especially bone and bone marrow metastases), age older than 10 years, a size larger than 200 ml, more central lesions (as in the pelvis or spine), and poor response to chemotherapy [3]. New pharmacological agents and radiotherapeutical modalities are being investigated as discussed in the earlier two articles in the symposium [49,50] and possibility of imporving the survival and quality of life of patients with ES looks promising.

Conclusion

ES is one of the common primary bone malignancies. Appropriate diagnosis, staging, biopsy and treatment at specialized centers is essential for a good outcome. Treatment is multi modal with neoadjuvant and adjuvant chemotherapy, surgery with appropriate margins and radiation in adjuvant or definitive setting; all playing important role in achieving good overall survival rates. Limb salvage surgery in non-metastatic ES is now a norm. The survivors are prone to many long-term complications and need to be followed up for a longer duration. .

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How to Cite this article: Panchwagh Y. Ewing Sarcoma: Focus on Surgical Management. Journal of  Bone and Soft Tissue Tumors May-Aug 2015;1(1):23-28.
Dr.Yogesh Panchwagh
Dr.Yogesh Panchwagh

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