Epithelioid sarcoma differential diagnosis

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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]

Overview

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

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

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