Tag Archive for: Surgery

Desmoplastic Fibroma of the Distal Femur in a Young Man: A Rare Case Report

Original Article | Volume 6 | Issue 3 | JBST September – December 2020 | Page 2-4 | Suresh Babu, Abhishek Vaish, Raju Vaishya DOI: 10.13107/jbst.2020.v06i03.30

Author: Suresh Babu[1], Abhishek Vaish[1], Raju Vaishya[1]

[1]Department of Orthopaedics and Joint Replacement Surgery, Indrparastha Apollo Hospitals, New Delhi, India.

Address of Correspondence
Dr. Suresh Babu,
Department of Orthopaedics and Joint Replacement Surgery, Indrparastha Apollo Hospitals, Sarita Vihar, New Delhi – 110 076, India.
E-mail: yssureshbabu@gmail.com

Introduction: Lytic lesions in the distal femur in a mature skeleton though a common presentation for various tumors, desmoplastic fibroma (DFB) of bone is a rare occurrence. Review of the literature shows its incidence of 0.06% to 0.3%. In the majority of reported cases, the diagnosis has been obtained on histopathological examination. Treatment varies from aggressive curettage to amputations. We describe a novel surgical technique for dealing such lesions.
Case Report: A 24-year-old male presented with swelling around with DFB the left distal femur presented with pain for 4 months which progressed in severity and led to inability to bear weight. On clinical examination, he had a tender discrete swelling over the medial aspect of the left distal femur. Radiographic examination showed an eccentric lytic lesion in the metaphyseoepiphyseal region of the left distal femur. An extended curettage using phenol (10%) as adjuvant therapy was performed. The cavity was packed with bone cement and the distal femur was fixed by spanning the lytic lesion with a distal femoral locking plate. At months follow-up, he reported complete resolution of symptoms, and on examination, he had pain free and full range of motion, without any signs of recurrence.
Conclusion: An effort should be made to obtain a pre-operative histopathological diagnosis of the tumor type. In cases of equivocal findings, a diagnosis of DFB of the bone should be considered. Extended curettage, phenol ablation, and bone cement with plate augmentation offer an effective treatment modality in the treatment of DFB.
Keywords: Desmoplastic fibroma, benign bone tumors, surgery, knee, femur.

Reference:
1. Campanacci M. Desmoid fibroma. In: Bone and Soft Tissue Tumors: Clinical Features, Imaging, Pathology and Treatment. Wien: Springer-Verlag; 1999. p. 143-8.
2. Campanacci L. Desmoid fibroma. In: Diagnosis of Musculoskeletal Tumors and Tumor-like Conditions. Berlin, Germany: Springer; 2019. p. 61-3.
3. Mazabraud A. Desmoid fibroma. In: Pathology of bone tumours. Springer, Berlin, Heidelberg; 1998;14:167-72 https://doi.org/10.1007/978-3-642-95839-7_14.
4. Kalil RK. Desmoplastic fibroma of bone. In: Tumors and Tumor-Like Lesions of Bone. Berlin, Germany: Springer; 2020. p. 451-7.
5. Inwards CY, Unni KK, Beabout JW, Sim FH. Desmoplastic fibroma of bone. Cancer 1991;68:1978-83.
6. Gebhardt MC, Campbell CJ, Schiller AL, Mankin HJ. Desmoplastic fibroma of bone: A report of eight cases and review of the literature. J Bone Joint Surg Am 1985;67:732-47.
7. Gao S, Cai Q, Yao W, Wang J, Zhang P, Wang X. Desmoplastic (collagenous) fibroma of the femur: A case report and review of the literature. Oncol Lett 2013;6:1285-8.
8. Gong LH, Liu WF, Ding Y, Geng YH, Sun XQ, Huang XY. Diagnosis and differential diagnosis of desmoplastic fibroblastoma by clinical, radiological, and histopathological analyses. Chin Med J 2018;131:32-6.
9. Crim JR, Gold RH, Mirra JM, Eckardt JJ, Bassett LW. Desmoplastic fibroma of bone: Radiographic analysis. Radiology 1989;172:827-32.
10. Xu Y, Wang Y, Yan J, Bai X, Xing G. Desmoplastic fibroma of the femur with atypical image findings: A case report. Medicine 2018;97:e13787.
11. Kang HS, Ahn JM, Kang Y. Radiographic findings. In: Oncologic Imaging: Bone Tumors. Berlin, Germany: Springer; 2017. p. 273-307.
12. Tanwar YS, Kharbanda Y, Rastogi R, Singh R. Desmoplastic fibroma of bone: A case series and review of literature. Indian J Surg Oncol 2018;9:585-91.
13. Nishida J, Tajima K, Abe M, Honda M, Inomata Y, Shimamura T, et al. Desmoplastic fibroma. Aggressive curettage as a surgical alternative for treatment. Clin Orthop Relat Res 1995;320:142-8.
14. Taconis WK, Schütte HE, van der Heul RO. Desmoplastic fibroma of bone: A report of 18 cases. Skeletal Radiol 1994;23:283-8.
15. Bohm P, Krober S, Greschniok A, Laniado M, Kaiserling E. Desmoplastic fibroma of the bone. A report of two patients, review of the literature, and therapeutic implications. Cancer 1996;78:1011-23.
16. Takazawa K, Tsuchiya H, Yamamoto N, Nonomura A, Suzuki M, Taki J, et al. Osteosarcoma arising from desmoplastic fibroma treated 16 years earlier: A case report. J Orthop Sci 2003;8:864-8.
17. Abdelwahab IF, Klein MJ, Hermann G, Steiner GC, Yang DC. Osteosarcoma arising in a desmoplastic fibroma of the proximal tibia. AJR Am J Roentgenol 2002;178:613-5.

How to Cite this article: Babu S, Vaishya A, Vaishya R | Desmoplastic Fibroma of the Distal Femur in a Young Man: A Rare Case Report. | Journal of Bone and Soft Tissue Tumors | Sep-Dec 2020; 6(3): 2-4.

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A Giant Parosteal Lipoma with Exostosis of the Right Proximal Humerus

Case Report | Volume 5 | Issue 2 | JBST May – August 2019 | Page 4-7| Abdaud Rasyid, Mujaddid Idulhaq, Pamudji Utomo, Ambar Mudigdo, Handry Tri Handojo. DOI: 10.13107/jbst.2019.v05i02.424

Author Abdaud Rasyid[1],[2], Mujaddid Idulhaq[1],[2], Pamudji Utomo[1],[2], Ambar Mudigdo[3], Handry Tri Handojo[4]

[1]Department of Orthopaedic and Traumatology, Faculty of Medicine, Sebelas Maret University, Surakarta, Indonesia,
[2]Department of Orthopaedic and Traumatology, Prof. DR. R. Soeharso Orthopaedic Hospital, Surakarta, Indonesia,
[3]Department of Anatomical Pathology, Sebelas Maret University, Surakarta, Indonesia,
[4]Department of Radiology, at Prof. DR. R. Soeharso Orthopaedic, Hospital, Surakarta, Indonesia.

Address of Correspondence
Dr. Abdaud Rasyid,
Jalan Ahmad Yani, Pabelan, Kartasura, Sukoharjo, Jawa Tengah, 57162, Indonesia.
E-mail: abdaudry@gmail.com

Abstract
Introduction: Lipomas are the most frequent benign soft-tissue tumors. Soft -tissue lipomas are categorized by anatomic location as either superficial (subcutaneous) or deep (intermuscular). Deep lipomas can be located in any part of the body, including the superior extremities. Lipomas typically reach a diameter of several centimeters and are localized in a single anatomical region. Parosteal lipoma is a rare subtype of deep lipoma that has a broad attachment to the underlying periosteum that forms an exostoses-like bone prominence. There has been no reliable literature; about two pathological processes occur in one extremity at the same time.
Case Presentation: A 49-years -old female presented at our institution with a painless, slow -growing lump in her right shoulder region since for 2 years ago, with no other symptoms, and no history of trauma. A palpable non-tender mobile mass was present on the right shoulder region. Plain radiographs showed a well-delineated ovoid radiolucent lesion and a radiopaque lesion over the right proximal humerus. The fine -needle biopsy result suggested a liposarcoma. Wide-excision surgery was performed for both the masses. On contrary, the histological examination of the specimen confirmed a giant lipoma with pieces of adult bone tissues.
Conclusion: Deep-seated lipomas are most commonly discovered in men between the ages of thirties 30s and sixties60s. In our patient, the lipoma also accompanied with an exostoses-like cartilaginous mass over the proximal humerus as in parosteal lipoma. Plain radiographs study of parosteal lipoma is associated with a false osteochondroma appearance, which also found in this patient. Histological examination suggested a giant lipoma for this patient, but the possibility of two pathological processes is still in question.
Keywords: Giant lipoma, shoulder, exostosis, surgery.

References
1. Slavchev S, Georgiev PP, Penkov M. Giant lipoma extending between the heads of biceps brachii muscle and the deltoid muscle: Case report. J Curr Surg 2012;2:146-8.
2. Stevenson J, Parry M. Tumours. In: Apley and Solomon’s System of Orthopaedics and Trauma, 10th ed. Vol. 9. Ch. 9. Florida: CRC Press; 2018. p. 223-4.
3. Singh V, Kumar V, Singh AK. Case report: A rare presentation of giant palmar lipoma. Int J Surg Case Rep 2016;26:21-3.
4. Elbardouni A, Kharmaz M, Salah Berrada M, Mahfoud M, Elyaacoubi M. Well-circumscribed deep-seated lipomas of the upper extremity. A report of 13 cases. Orthop Traumatol Surg Res 2011;97:152-8.

How to Cite this article: Rasyid A, Idulhaq M, Utomo P, Mudigdo A, Handojo H T. A Giant Parosteal Lipoma with Exostosis of the Right Proximal Humerus. Journal of Bone and Soft Tissue Tumors May-August 2019;5(2): 4-7.

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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. .

References

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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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