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Langerhans Cell Histiocytosis of the Spine in a Child: A Rare Case and a Diagnostic Dilemma

September 10, 2020

Original Article | Volume 6 | Issue 3 | JBST September – December 2020 | Page 5-8 | Abigail R Tud, Jose Bayani O Aliling, Carlo Emmanuel J SumpaicoDOI: 10.13107/jbst.2020.v06i03.31 Author: Abigail R Tud[1], Jose Bayani O Aliling[1], Carlo Emmanuel J Sumpaico[1] [1]Department of Orthopedics, The Medical City, Ortigas Avenue, Pasig City, Metro Manila 1605, […]

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

September 10, 2020

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 […]

Langerhans Cell Histiocytosis of the Spine in a Child: A Rare Case and a Diagnostic Dilemma

September 10, 2020

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

September 10, 2020

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Langerhans Cell Histiocytosis of the Spine in a Child: A Rare Case and a Diagnostic Dilemma

September 10, 2020

Author: Abigail R Tud[1], Jose Bayani O Aliling[1], Carlo Emmanuel J Sumpaico[1]

[1]Department of Orthopedics, The Medical City, Ortigas Avenue, Pasig City, Metro Manila 1605, Philippines.

Address of Correspondence
Dr. Abigail R Tud,
Unit 5F Tower 1, Sonata Private Residences, Lourdes corner St. Francis Avenues, East Wack Wack, Mandaluyong City, Metro Manila 1505, Philippines.
E-mail address: abitud@gmail.com

Introduction: Langerhans cell histiocytosis (LCH) comprises a rare spectrum of disorders characterized by abnormal proliferation of histiocytes. Lesions may be limited to a single system, or present as disseminated disease, with subsequent worse prognosis. Radiologic findings for spinal LCH are non-specific however and must be carefully differentiated from Pott’s disease (spinal tuberculosis, or [TB]) in endemic countries.
Case Report: An 8-year-old female was brought for consult due to back pain and compression deformity on plain radiographs, unaccompanied by constitutional symptoms. Computed tomography (CT) scan of the thoracic spine was done, which showed osteolysis of the T11 vertebra and paravertebral soft-tissue extension. Findings were deemed suggestive of tuberculous spondylitis, and CT-guided biopsy was performed for confirmation. Acid-fast Bacilli smears as well as Gram stains were negative. While waiting for definitive results, the patient was started on an empiric treatment regimen for TB. Final tissue and fluid cultures were negative for Mycobacterium tuberculosis, as were Gene Xpert and TB polymerase chain reaction studies. Histopathologic analysis showed atypical mononuclear histiocytes surrounded by inflammatory cells, suggestive of LCH. At present, the patient is 2 years post-biopsy, with stable lesions and no evidence of multi-system involvement.
Conclusion: In the setting of a spinal lesion in a child with a benign clinical history and no other pertinent laboratory findings, histopathologic analysis constitutes the gold standard in differentiating non-specific features of LCH from spinal TB. Both conditions necessitate long-term follow-up, due to the risk of progression, deformities, and neurologic sequelae.
Keywords: Langerhans cell histiocytosis, pediatric spine, Pott’s disease, tuberculosis, vertebra plana.

Reference:
1. Imbach P, Kühne T, Arceci RJ. Histiocytoses. In: Pediatric Oncology: A Comprehensive Guide. United States: Springer Science and Business Media; 2011.
2. Lee SW, Kim H, Suh JK, Koh KN, Im HJ, Yoon HM, et al. Long-term clinical outcome of spinal Langerhans cell histiocytosis in children. Int J Hematol 2017;106:441-9.
3. Stålemark H, Laurencikas E, Karis J, Gavhed D, Fadeel B, Henter JI. Incidence of Langerhans cell histiocytosis in children: A population-based study. Pediatr Blood Cancer 2008;51:76-81.
4. Jang KS, Jung YY, Kim SW. Langerhans cell histiocytosis causing cervical myelopathy in a child. J Korean Neurosurg Soc 2010;47:458-60.
5. Peng XS, Pan T, Chen LY, Huang G, Wang J. Langerhans’ cell histiocytosis of the spine in children with soft tissue extension and chemotherapy. Int Orthop 2009;33:731-6.
6. Fellizar KR, Chiong CM. Langerhans cell histiocytosis: An unusual presentation. Philipp J Otolaryngol Head Neck Surg 2008;23:20-4.
7. Garg RK, Somvanshi DS. Spinal tuberculosis: A review. J Spinal Cord Med 2011;34:440-54.
8. Haupt R, Minkov M, Astigarraga I, Schäfer E, Naduri V, Jubran R, et al. Langerhans cell histiocytosis (lch): Guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years. Pediatr Blood Cancer 2013;60:175-84.
9. Huang, WD, Yang XH, Wu ZP, Huang Q, Xiao JR, Yang MS, et al. Langerhans cell histiocytosis of spine: A comparative study of clinical, imaging features, and diagnosis in children, adolescents, and adults. Spine J 2013;3:1-10.
10. Stull MA, Krandsdorf MJ, Devaney KO. Langerhans cell histiocytosis. Radiographics 1992;12:801-25.
11. Department of Health/National TB Control Program Philippine Council for Health Research and Development Foundation for the Advancement of Clinical Epidemiology, Inc. Available from: https://www.philcat.org. [Last accessed on 2018 Sep 29].
12. Xing XY, Yuan HS. Imaging and differential diagnosis of pediatric spinal tuberculosis. Radiol Infect Dis 2015;1:78-82.
13. Rasouli MR, Mirkoohi M, Vaccaro AR, Yarandi KK, Rahimi-Movaghar V. Spinal tuberculosis: Diagnosis and management. Asian Spine J 2012;6:294-308.
14. Gannaban RA, Calimag PP, Zapanta ZS. Langerhans cell histiocystosis presenting as a scalp abscess: A case report. PJSS 2011;66:74-9.
15. Quijano JL, Esguerra AK. Single-system Langerhans cell histiocytosis in a newborn male with positive Tzanck smear. J Philipp Dermatol Soc 2016;25:51-3.
16. Rigor E, Delos Reyes E, Lecciones J, Purugganan H, Fajardo RV. Langerhans’ cell histiocytosis (histiocytosis X) a 12-year retrospective study. Philipp J Pediatr 1993;42:181-9.
17. Villegas MJ, Ke ML. Langerhans’ cell histiocytosis presenting as ulcers. J Philipp Soc Cutan Med 2000;1:90-4.

How to Cite this article: Tud AR, Aliling JBO, Sumpaico CEJ | Langerhans Cell Histiocytosis of the Spine in a Child: A Rare Case and a Diagnostic Dilemma. | Journal of Bone and Soft Tissue Tumors | September-December 2020; 6(3): 5-8.

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

September 10, 2020

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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