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Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.

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Epithelioid sarcoma differential diagnosis

Introduction

Overview

Overview

Epithelioid sarcoma must be differentiated from synovial sarcoma, wart, ganglion cysts, rhabdomyosarcoma, and clear cell sarcoma.

Differentiating Epithelioid sarcoma from other Diseases

Differentiating Epithelioid sarcoma from other Diseases

Keratin CD34 Cytoplasm In-sutu Kerataniztion Location Age group H/O of epithelial injury CA125 CK5/6 GCDFP15, S100, actin Necrosis Calcification Hemangiopericytomatous vessels
Epitheloid Sarcoma Positive Positive Sharply defined Negative Hand and wrist Under 40 years No Positive Rare, Local Negative Extensive Infrequent Negative
Palisaded Necrotic Granuloma Neagtive Negative Indistinct Uncommon
Squamous Carcinoma Negative Positive >40 Positive Negative in cutaneous SCC

Positive everywhere

Extensive
Sweat Gland Carcinoma Negative Positive
Monophasic Synovial Sarcoma Negative Negative Focal Frequent Positive
Melanoma Negative Negative Negative Positive
Epithelioid Hemangioendothelioma Negative Uncommon on extremities
Sclerosing Epithelioid Fibrosarcoma Negative Cords of cells in hylainzed stroma
Ischemic Fasciitis Negative Basophilic or amphophilic
Disease History/demography Symptoms Physical examination Diagnosis
Palpable mass Pain Others Mass tenderness Others Genetics Imaging Histology
Epitheloid Sarcoma
  • Epithelioid sarcoma is a very rare disease.
  • The incidence of epithelioid sarcoma increases between ages of 10 and 39 and the average age at presentation is 27 years.
  • Males are more commonly affected with epithelioid sarcoma than females. Epithelioid sarcoma of the upper extremity usually affects individuals of the Caucasian race.
 + – Mainly cutaneous manifestations –
  • Irregular (or nodular) lesions below the skin surface
  • Lesion with the appearance of a badly-healed wound
  • Loss of INI1 ocated on the long arm of chromosome 22 (22q11.2).
  • On T2/fluid sequences, the mass is typically hyperintense with areas of central necrosis and surrounding edema.
  • There is usually heterogeneous enhancement, often with areas of necrosis.
  • A characteristic finding, is propagation along fascial planes, tendon sheaths and nerves.
  • Radiography and CT may reveal a soft-tissue mass with speckled calcifications.
  • There may be adjacent osseous remodeling; however, cortical destruction and invasion are rare.
  • Tissue biopsy is the diagnostic modality of choice for epithelioid sarcoma.
  • White nodules with infiltrating margins
  • Epithelial cells well blended with fusiform cells with intracytoplasmic vacuoles
  • “Pseudogranulomatous” proliferation of cells around acellular necrotic debris
  • Hyalinized collagen and necrotic debris in the central zone
  • Calcification in the necrotic zones
  • Multinuclear giant cells in the tumors
Rhabdomyosarcoma[2][3][4][5]
  • Most common soft tissue cancer among children and adolescents
  • The third most common extracranial solid tumors
  • Two-third of all cases happen under 6 years old
 + + +/-

Mutations in:

CT scan:

Ultrasound:

MRI:

Wilms tumor[6][7][8][9][10] + + +/- Present mutations of: Ultrasound:

CT scan:

Ewing sarcoma[11][12][13][14] + + + Radiographic of region:

CT scan:

MRI:

Pediatric neuroblastoma [15][16][17][18]

Age distribution:

  • < 1 years old ( 40%)
  • 1-2 years old (35%)
  • > 2 years old (25%)

+ (Abdominal)

+

+(Abdominal)

CT scan:

MRI:

Pediatric pheochromocytoma[19][20][21][22] – +/- – Genetic mutation in: Ultrasound:

CT scan:

MRI (in extra adrenal tumors):

  • T1:
    • Heterogenous enhancement
    • Hypointense
  • T2:
    • Hyperintense
  • T1 C+ (Gd):
    • Heterogenous enhancement

Positive stains for:

Pediatric osteosarcoma[23][24][25] + + + Radiography:

CT scan:

MRI:

Pediatric liposarcoma[26][27][28][29] + +/- –
  • N/A
 CT scan:

MRI:

Divided into following subtypes:

Common findings:

Pediatric acute myelocytic leukemia[30][31][32][33] +/- ( Abdominal mass, mediastinal mass) + (bone pain, joint pain) +/- Genetic translocations include:
  • t (8;21)
  • t (3;21)
  • t (15;17)
 Radiography:

CT scan/ MRI:

Radionuclide imaging:

Pediatric acute lymphoblastic leukemia[34][35]

+/-( Extramedullary mass in abdomen/ head/ neck)

+/- (Musculoskeletal pain) – Chromosomal translocations:
  • t (9;22)
  • t (12;21)
  • t (5;14)
  • t (1;19)
 Radiography:

Chest x ray:

Bone x ray:

Brain MRI:

Divided into 3 subgroups:

L1:

L2:

L3:

Pediatric non-hodgkin lymphoma[36][37][38] + – + (Chest tenderness) Radiography:

CT scan:

Ultrasound:

Histology findings of non-hodgkin lymphoma depend on:
References

References

  1. ↑ Epithelioid sarcoma. Sarcomahelp (2016). http://sarcomahelp.org/epithelioid-sarcoma.html Accessed on February 8, 2016
  2. ↑ Egas-Bejar D, Huh WW (2014). “Rhabdomyosarcoma in adolescent and young adult patients: current perspectives”. Adolesc Health Med Ther. 5: 115–25. doi:10.2147/AHMT.S44582. PMC 4069040. PMID 24966711.
  3. ↑ Dasgupta R, Fuchs J, Rodeberg D (2016). “Rhabdomyosarcoma”. Semin Pediatr Surg. 25 (5): 276–283. doi:10.1053/j.sempedsurg.2016.09.011. PMID 27955730.
  4. ↑ Park K, van Rijn R, McHugh K (2008). “The role of radiology in paediatric soft tissue sarcomas”. Cancer Imaging. 8: 102–15. doi:10.1102/1470-7330.2008.0014. PMC 2365455. PMID 18442956.
  5. ↑ Shern JF, Yohe ME, Khan J (2015). “Pediatric Rhabdomyosarcoma”. Crit Rev Oncog. 20 (3–4): 227–43. PMC 5486973. PMID 26349418.
  6. ↑ Hartman DS, Sanders RC (April 1982). “Wilms’ tumor versus neuroblastoma: usefulness of ultrasound in differentiation”. J Ultrasound Med. 1 (3): 117–22. PMID 6152936.
  7. ↑ De Campo JF (1986). “Ultrasound of Wilms’ tumor”. Pediatr Radiol. 16 (1): 21–4. PMID 3003660.
  8. ↑ Cahan LD (1985). “Failure of encephalo-duro-arterio-synangiosis procedure in moyamoya disease”. Pediatr Neurosci. 12 (1): 58–62. PMID 4080660.
  9. ↑ Coppes MJ, Pritchard-Jones K (2000). “Principles of Wilms’ tumor biology”. Urol Clin North Am. 27 (3): 423–33, viii. PMID 10985142.
  10. ↑ Davidoff AM (2012). “Wilms tumor”. Adv Pediatr. 59 (1): 247–67. doi:10.1016/j.yapd.2012.04.001. PMC 3589819. PMID 22789581.
  11. ↑ Burchill SA (2003). “Ewing’s sarcoma: diagnostic, prognostic, and therapeutic implications of molecular abnormalities”. J Clin Pathol. 56 (2): 96–102. PMC 1769883. PMID 12560386.
  12. ↑ Maygarden SJ, Askin FB, Siegal GP, Gilula LA, Schoppe J, Foulkes M; et al. (1993). “Ewing sarcoma of bone in infants and toddlers. A clinicopathologic report from the Intergroup Ewing’s Study”. Cancer. 71 (6): 2109–18. PMID 8443760.
  13. ↑ Panicek DM, Gatsonis C, Rosenthal DI, Seeger LL, Huvos AG, Moore SG; et al. (1997). “CT and MR imaging in the local staging of primary malignant musculoskeletal neoplasms: Report of the Radiology Diagnostic Oncology Group”. Radiology. 202 (1): 237–46. doi:10.1148/radiology.202.1.8988217. PMID 8988217.
  14. ↑ Grünewald TGP, Cidre-Aranaz F, Surdez D, Tomazou EM, de Álava E, Kovar H; et al. (2018). “Ewing sarcoma”. Nat Rev Dis Primers. 4 (1): 5. doi:10.1038/s41572-018-0003-x. PMID 29977059.
  15. ↑ Lonergan GJ, Schwab CM, Suarez ES, Carlson CL (2002). “Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation”. Radiographics. 22 (4): 911–34. doi:10.1148/radiographics.22.4.g02jl15911. PMID 12110723.
  16. ↑ Golden CB, Feusner JH (2002). “Malignant abdominal masses in children: quick guide to evaluation and diagnosis”. Pediatr Clin North Am. 49 (6): 1369–92, viii. PMID 12580370.
  17. ↑ Angstman KB, Miser JS, Franz WB (1990). “Neuroblastoma”. Am Fam Physician. 41 (1): 238–44. PMID 2403727.
  18. ↑ Musarella MA, Chan HS, DeBoer G, Gallie BL (1984). “Ocular involvement in neuroblastoma: prognostic implications”. Ophthalmology. 91 (8): 936–40. PMID 6493702.
  19. ↑ Leung K, Stamm M, Raja A, Low G (2013). “Pheochromocytoma: the range of appearances on ultrasound, CT, MRI, and functional imaging”. AJR Am J Roentgenol. 200 (2): 370–8. doi:10.2214/AJR.12.9126. PMID 23345359.
  20. ↑ Stein PP, Black HR (1991). “A simplified diagnostic approach to pheochromocytoma. A review of the literature and report of one institution’s experience”. Medicine (Baltimore). 70 (1): 46–66. PMID 1988766.
  21. ↑ Bravo EL (1991). “Pheochromocytoma: new concepts and future trends”. Kidney Int. 40 (3): 544–56. PMID 1787652.
  22. ↑ Bravo EL (1991). “Pheochromocytoma: new concepts and future trends”. Kidney Int. 40 (3): 544–56. PMID 1787652.
  23. ↑ Dorfman HD, Czerniak B (1995). “Bone cancers”. Cancer. 75 (1 Suppl): 203–10. PMID 8000997.
  24. ↑ Yarmish G, Klein MJ, Landa J, Lefkowitz RA, Hwang S (2010). “Imaging characteristics of primary osteosarcoma: nonconventional subtypes”. Radiographics. 30 (6): 1653–72. doi:10.1148/rg.306105524. PMID 21071381.
  25. ↑ Araki N, Uchida A, Kimura T, Yoshikawa H, Aoki Y, Ueda T; et al. (1991). “Involvement of the retinoblastoma gene in primary osteosarcomas and other bone and soft-tissue tumors”. Clin Orthop Relat Res (270): 271–7. PMID 1884549.
  26. ↑ Shmookler BM, Enzinger FM (1983). “Liposarcoma occurring in children. An analysis of 17 cases and review of the literature”. Cancer. 52 (3): 567–74. PMID 6861094.
  27. ↑ Marcus KC, Grier HE, Shamberger RC, Gebhardt MC, Perez-Atayde A, Silver B; et al. (1997). “Childhood soft tissue sarcoma: a 20-year experience”. J Pediatr. 131 (4): 603–7. PMID 9386667.
  28. ↑ Murphey MD, Arcara LK, Fanburg-Smith J (2005). “From the archives of the AFIP: imaging of musculoskeletal liposarcoma with radiologic-pathologic correlation”. Radiographics. 25 (5): 1371–95. doi:10.1148/rg.255055106. PMID 16160117.
  29. ↑ Italiano A, Cardot N, Dupré F, Monticelli I, Keslair F, Piche M; et al. (2007). “Gains and complex rearrangements of the 12q13-15 chromosomal region in ordinary lipomas: the “missing link” between lipomas and liposarcomas?”. Int J Cancer. 121 (2): 308–15. doi:10.1002/ijc.22685. PMID 17372913.
  30. ↑ Yamamoto JF, Goodman MT (2008). “Patterns of leukemia incidence in the United States by subtype and demographic characteristics, 1997-2002”. Cancer Causes Control. 19 (4): 379–90. doi:10.1007/s10552-007-9097-2. PMID 18064533.
  31. ↑ Cancer Genome Atlas Research Network. Ley TJ, Miller C, Ding L, Raphael BJ, Mungall AJ; et al. (2013). “Genomic and epigenomic landscapes of adult de novo acute myeloid leukemia”. N Engl J Med. 368 (22): 2059–74. doi:10.1056/NEJMoa1301689. PMC 3767041. PMID 23634996.
  32. ↑ Islam A, Catovsky D, Goldman JM, Galton DA (1985). “Bone marrow biopsy changes in acute myeloid leukaemia. I: Observations before chemotherapy”. Histopathology. 9 (9): 939–57. PMID 3864727.
  33. ↑ Orazi A (2007). “Histopathology in the diagnosis and classification of acute myeloid leukemia, myelodysplastic syndromes, and myelodysplastic/myeloproliferative diseases”. Pathobiology. 74 (2): 97–114. doi:10.1159/000101709. PMID 17587881.
  34. ↑ Zuckerman T, Rowe JM (2014). “Pathogenesis and prognostication in acute lymphoblastic leukemia”. F1000Prime Rep. 6: 59. doi:10.12703/P6-59. PMC 4108947. PMID 25184049.
  35. ↑ Pui CH, Robison LL, Look AT (2008). “Acute lymphoblastic leukaemia”. Lancet. 371 (9617): 1030–43. doi:10.1016/S0140-6736(08)60457-2. PMID 18358930.
  36. ↑ Green MR, Gentles AJ, Nair RV, Irish JM, Kihira S, Liu CL; et al. (2013). “Hierarchy in somatic mutations arising during genomic evolution and progression of follicular lymphoma”. Blood. 121 (9): 1604–11. doi:10.1182/blood-2012-09-457283. PMC 3587323. PMID 23297126.
  37. ↑ Sandlund JT (2015). “Non-Hodgkin Lymphoma in Children”. Curr Hematol Malig Rep. 10 (3): 237–43. doi:10.1007/s11899-015-0277-y. PMID 26174528.
  38. ↑ El-Galaly TC, Hutchings M (2015). “Imaging of non-Hodgkin lymphomas: diagnosis and response-adapted strategies”. Cancer Treat Res. 165: 125–46. doi:10.1007/978-3-319-13150-4_5. PMID 25655608.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]