Soft Tissue and Bone Tumors

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First Online: 15 June 2022

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Handbook of Practical Immunohistochemistry

Soft Tissue and Bone Tumors

George Lin⁵ &
Shaobo Zhu⁶

2120 Accesses

Abstract

Immunohistochemistry is a powerful adjunctive technique for the pathologic diagnosis of soft tissue and bone tumors, although some tumors still lack specific markers. This chapter includes questions about the immunohistochemical markers for normal soft tissue and bone, soft tissue and bone tumors, and their utility for differentiation. The questions are answered in the form of tables. The photos of selected markers are also included. Newer markers such as BCOR, CCNB3, claudin-5, EVT4, ERG, GAP43, H3G34W, H3K36M, H3K27me3, INI1, INSM1, MDM2, MUC4, NKX2.2, NY-ESO-1, retinoblastoma, SATB2, SOX9, SOX10, STAT6, SS18-SSX as well as other commonly used markers are discussed.

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Keywords

Frequently Asked Questions

1.
Summary of applications and limitations of useful markers (Table 30.1)
2.
Markers positive for normal soft tissue and bone (Table 30.2)

Adipocytic Tumors

3.
Markers for spindle cell lipoma/pleomorphic lipoma (Table 30.3)
4.
Markers for well-differentiated liposarcoma/dedifferentiated liposarcoma (Table 30.4)
5.
Markers for myxoid/round cell liposarcoma (Table 30.5)

Fibroblastic and Fibrohistiocytic Tumors

6.
Markers for nodular fasciitis (Table 30.6)
7.
Markers for palmar and plantar (superficial) fibromatosis (Table 30.7)
8.
Markers for deep fibromatosis (Table 30.8)
9.
Markers for fibrous hamartoma of infancy (Table 30.9 )
10.
Markers for inflammatory myofibroblastic tumor/inflammatory fibrosarcoma (Table 30.10)
11.
Markers of myofibroma/myofibromatosis (Table 30.11)
12.
Markers for angiomyofibroblastoma (Table 30.12)
13.
Markers for cellular angiofibroma (Table 30.13)
14.
Markers of mammary-type myofibroblastoma (Table 30.14)
15.
Markers for myxoinflammatory fibroblastic sarcoma (Table 30.15)
16.
Markers for low-grade myofibroblastic sarcoma (Table 30.16)
17.
Markers for low-grade fibromyxoid sarcoma including spindle cell tumor with giant rosettes (Table 30.17)
18.
Markers for myxofibrosarcoma (Table 30.18)

Fibrohistiocytic Tumors

19.
Markers for benign fibrous histiocytoma (Table 30.19)
20.
Markers for juvenile xanthogranuloma and reticulohistiocytoma (Table 30.20)
21.
Markers for atypical fibroxanthoma (Table 30.21)
22.
Markers for dermatofibrosarcoma protuberans/giant cell fibroblastoma (Table 30.22)
23.
Markers for angiomatoid fibrous histiocytoma (Table 30.23)
24.
Markers for plexiform fibrohistiocytic tumor (Table 30.24)
25.
Markers for giant cell tumor of soft tissue (Table 30.25)
26.
Markers for undifferentiated pleomorphic sarcoma (pleomorphic malignant fibrous histiocytoma) (Table 30.26)

Smooth Muscle Tumors

27.
Markers for leiomyoma and leiomyosarcoma (Table 30.27)
28.
Markers for EBV-related leiomyosarcoma (Table 30.28)

Skeletal Muscle Tumors

29.
Markers for rhabdomyoma (Table 30.29)
30.
Markers for rhabdomyosarcoma (Table 30.30)

Tumors of Perivascular Cells

31.
Markers for hemangiopericytoma (HPC)/solitary fibrous tumor (SFT) of soft tissue (Table 30.31)
32.
Markers for myopericytoma family of tumors (infantile myofibromatosis, solitary myofibroma, infantile hemangiopericytoma, myopericytoma, and glomangiopericytoma) (Table 30.32)
33.
Markers for glomus tumor (Table 30.33)

Vascular Tumors

34.
Markers for kaposiform hemangioendothelioma (Table 30.34)
35.
Markers for hemangioma (Table 30.35)
36.
Markers for Dabska-type and retiform hemangioendothelioma (Table 30.36)
37.
Markers for epithelioid hemangioendothelioma (Table 30.37)
38.
Markers for angiosarcoma (Table 30.38)
39.
Markers for Kaposi sarcoma (Table 30.39)
40.
Markers for lymphatic tumors (Table 30.40)

Nerve Sheath and Neuroectodermal Tumors

41.
Markers for neurofibroma (Table 30.41)
42.
Markers for schwannoma/nerve sheath myxoma (Table 30.42)
43.
Markers for psammomatous melanotic schwannoma/ malignant melanotic schwannian tumor (Table 30.43)
44.
Markers for malignant peripheral nerve sheath tumor (MPNST) (Table 30.44)
45.
Markers for granular cell tumor (Table 30.45)
46.
Markers for neurothekeomas (Table 30.46)
47.
Markers for perineurioma (Table 30.47)
48.
Markers for neuroblastoma (Table 30.48)
49.
Markers for clear cell sarcoma of soft parts (Table 30.49)

Tumors of Uncertain Differentiation

50.
Markers for myxoma (Table 30.50)
51.
Markers for aggressive angiomyxoma (Table 30.51)
52.
Markers for ossifying fibromyxoid tumor of soft parts (Table 30.52)
53.
Markers for myoepithelial tumors (mixed tumor/myoepithelioma/parachordoma) (Table 30.53)
54.
Markers for pleomorphic hyalinizing angiectatic tumor (Table 30.54)
55.
Markers for phosphaturic mesenchymal tumor (Table 30.55)
56.
Markers for perivascular epithelioid cell neoplasms (angiomyolipoma of the kidney or other organs, clear cell sugar tumor of the lung, lymphangioleiomyomatosis, clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres) (Table 30.56)
57.
Markers for epithelioid sarcoma (Table 30.57)
58.
Markers for alveolar soft part sarcoma (Table 30.58)
59.
Markers for Ewing sarcoma and primitive neuroectodermal tumor (ES/PNET) (Table 30.59)
60.
Markers for synovial sarcoma (Table 30.60)
61.
Markers for desmoplastic small round cell tumor (Table 30.61)
62.
Markers for malignant extrarenal rhabdoid tumor (Table 30.62)
63.
CIC-rearranged sarcoma (Table 30.63)
64.
BCOR-rearranged sarcoma (Table 30.64)
65.
Markers for extraskeletal myxoid chondrosarcoma (Table 30.65)

Bone Tumors

66.
Markers for mesenchymal chondrosarcoma (Table 30.66)
67.
Markers for chordoma (Table 30.67)
68.
Markers for adamantinoma (Table 30.68)
69.
Markers for Langerhans cell histiocytosis (Table 30.69)
70.
Markers for giant cell tumor of bone (Table 30.70)
71.
Markers for chondroblastoma (Table 30.71)

Differential Diagnosis

72.
Inflammatory myofibroblastic tumor (IMT)/inflammatory fibrosarcoma (IF) versus dendritic cell neoplasms (DCN) (Table 30.72)
73.
Inflammatory myofibroblastic tumor (IMT)/inflammatory fibrosarcoma (IF) versus sarcomatoid urothelial carcinoma (SUC) (Table 30.73)
74.
Myxoinflammatory fibroblastic sarcoma (MFS) versus Hodgkin’s lymphoma (HL) (Table 30.74)
75.
Fibrosarcoma (CF) versus monomorphic synovial sarcoma (MSS) versus malignant peripheral nerve sheath tumor (MPNST) versus spindle cell carcinoma (SCC) versus spindle cell melanoma (SCM) versus dermatofibrosarcoma protuberans (DFSP) versus spindle cell rhabdomyosarcoma (SCR) versus leiomyosarcoma (LMS) versus spindle cell angiosarcoma (SCA) (Table 30.75)
76.
Low-grade fibromyxoid sarcoma (LGFS) versus neurofibroma/perineurioma versus desmoid tumors (Table 30.76)
77.
Juvenile xanthogranuloma (JXG)/reticulohistiocytoma (RC) versus Langerhans cell histiocytosis (LCH) (Table 30.77)
78.
Dermatofibroma protuberans (DFSP) versus dermatofibroma (DF) versus fibrosarcoma arising in DFSP (Table 30.78)
79.
Rhabdomyosarcoma (RMS) versus Ewing sarcoma/PNET (ES/PNET) versus neuroblastoma (NB) versus desmoplastic small round cell tumor (DSRCT) versus small cell synovial sarcoma (SCSS) versus lymphoma (Table 30.79)
80.
Leiomyoma versus GIST versus schwannoma (Table 30.80)
81.
Glomus tumors (GT) versus hemangiopericytoma (HPC)/solitary fibrous tumor (SFT) of soft tissue (Table 30.81)
82.
Kaposiform hemangioendothelioma (KH) versus capillary hemangioma (CH) (Table 30.82)
83.
Neurofibroma versus schwannoma versus perineurioma (Table 30.83)
84.
ES/PNET versus lymphoblastic lymphoma (Table 30.84)
85.
ES/PNET versus neuroblastoma (Table 30.85)
86.
Chordoma versus chondrosarcoma (Table 30.86)
87.
Chordoma versus renal cell carcinoma (Table 30.87)
88.
Langerhans cell histiocytosis (LCH) versus Hodgkin lymphoma (HL) (Table 30.88)
89.
Epithelioid sarcoma (ES) versus epithelioid angiosarcoma (EA) versus epithelioid malignant peripheral nerve sheath tumor (EPNST) versus epithelioid leiomyosarcoma (ELMS) versus sclerosing epithelioid fibrosarcoma (SEF) versus epithelioid osteosarcoma (EO) (Table 30.89)
90.
Rhabdomyoma versus granular cell tumor (Table 30.90)
91.
Rhabdomyosarcoma versus leiomyosarcoma (Table 30.91)

Table 30.1 Summary of applications and limitations of useful markers

Full size table

Table 30.2 Markers positive for normal soft tissue and bone

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Adipocytic Tumors

Table 30.3 Markers for spindle cell lipoma/pleomorphic lipoma

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Table 30.4 Markers for well-differentiated liposarcoma/dedifferentiated liposarcoma

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Table 30.5 Markers for myxoid/round cell liposarcoma

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Fibroblastic and Fibrohistiocytic Tumors

Table 30.6 Markers for nodular fasciitis

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Table 30.7 Markers for palmar and plantar (superficial) fibromatosis

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Table 30.8 Markers for deep fibromatosis

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Table 30.9 Markers for fibrous hamartoma of infancy

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Table 30.10 Markers for inflammatory myofibroblastic tumor/inflammatory fibrosarcoma

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Table 30.11 Markers of myofibroma/myofibromatosis

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Table 30.12 Markers for angiomyofibroblastoma

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Table 30.13 Markers for cellular angiofibroma

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Table 30.14 Markers of mammary-type myofibroblastoma

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Table 30.15 Markers for myxoinflammatory fibroblastic sarcoma

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Table 30.16 Markers for low-grade myofibroblastic sarcoma

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Table 30.17 Markers for low-grade fibromyxoid sarcoma including spindle cell tumor with giant rosettes

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Table 30.18 Markers for myxofibrosarcoma

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Fibrohistiocytic Tumors

Table 30.19 Markers for benign fibrous histiocytoma

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Table 30.20 Markers for juvenile xanthogranuloma and reticulohistiocytoma

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Table 30.21 Markers for atypical fibroxanthoma

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Table 30.22 Markers for dermatofibrosarcoma protuberans/giant cell fibroblastoma

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Table 30.23 Markers for angiomatoid (malignant) fibrous histiocytoma

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Table 30.24 Markers for plexiform fibrohistiocytic tumor

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Table 30.25 Markers for giant cell tumor of soft tissue

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Table 30.26 Markers for undifferentiated pleomorphic sarcoma (pleomorphic malignant fibrous histiocytoma)

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Smooth Muscle Tumors

Table 30.27 Markers for leiomyoma and leiomyosarcoma (Fig. 30.1)

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Fig. 30.1

Table 30.28 Markers for EBV-associated leiomyosarcoma

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Skeletal Muscle Tumors

Table 30.29 Markers for rhabdomyoma

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Table 30.30 Markers for rhabdomyosarcoma (Fig. 30.2)

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Fig. 30.2

Tumors of Perivascular Cells

Table 30.31 Markers for hemangiopericytoma (HPC)/solitary fibrous tumor (SFT) of soft tissue (Fig. 30.3)

Full size table

Fig. 30.3

Table 30.32 Markers for myopericytoma family of tumors (infantile myofibromatosis, solitary myofibroma, infantile hemangiopericytoma myopericytoma, glomangiopericytoma)

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Table 30.33 Markers for glomus tumor (Fig. 30.4)

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Fig. 30.4

Vascular Tumors

Table 30.34 Markers for kaposiform hemangioendothelioma

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Table 30.35 Markers for hemangioma

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Table 30.36 Markers for Dabska-type and retiform hemangioendothelioma

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Table 30.37 Markers for epithelioid hemangioendothelioma

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Table 30.38 Markers for angiosarcoma (Fig. 30.5)

Full size table

Fig. 30.5

Table 30.39 Markers for Kaposi sarcoma

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Table 30.40 Markers for lymphatic tumors

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Nerve Sheath and Neuroectodermal Tumors

Table 30.41 Markers for neurofibroma

Full size table

Table 30.42 Markers for schwannoma/nerve sheath myxoma (Fig. 30.6)

Full size table

Fig. 30.6

Table 30.43 Markers for melanotic schwannoma

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Table 30.44 Markers for malignant peripheral nerve sheath tumor (MPNST)

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Table 30.45 Markers for granular cell tumor

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Table 30.46 Markers for cellular neurothekeomas (fibrohistiocytic tumor)

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Table 30.47 Markers for perineurioma

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Table 30.48 Markers for neuroblastoma (Fig. 30.7)

Full size table

Fig. 30.7

Table 30.49 Markers for clear cell sarcoma of soft parts

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Table 30.50 Markers for myxoma

Full size table

Table 30.51 Markers for aggressive angiomyxoma

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Table 30.52 Markers for ossifying fibromyxoid tumor of soft parts

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Table 30.53 Markers for mixed tumor/myoepithelioma/parachordoma (myoepithelial tumors)

Full size table

Table 30.54 Markers for pleomorphic hyalinizing angiectatic tumor

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Table 30.55 Markers for phosphaturic mesenchymal tumor

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Table 30.56 Markers for perivascular epithelioid cell family of tumors (PEComas) (angiomyolipoma of the kidney or other organs, clear cell sugar tumor of the lung, lymphangioleiomyomatosis, clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres)

Full size table

Table 30.57 Markers for epithelioid sarcoma (Fig. 30.8)

Full size table

Fig. 30.8

Table 30.58 Markers for alveolar soft part sarcoma

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Table 30.59 Markers for Ewing sarcoma (Fig. 30.9)

Full size table

Fig. 30.9

Table 30.60 Markers for synovial sarcoma (Fig. 30.10)

Full size table

Fig. 30.10

Table 30.61 Markers for desmoplastic small round cell tumor (Fig. 30.11)

Full size table

Fig. 30.11

Table 30.62 Markers for malignant extrarenal rhabdoid tumor

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Table 30.63 Markers for CIC-rearranged sarcoma

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Table 30.64 Markers for BCOR-rearranged sarcoma

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Table 30.65 Markers for extraskeletal myxoid chondrosarcoma

Full size table

Table 30.66 Markers for mesenchymal chondrosarcoma

Full size table

Table 30.67 Markers for chordoma

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Table 30.68 Markers for adamantinoma

Full size table

Table 30.69 Markers for Langerhans cell histiocytosis

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Table 30.70 Markers for giant cell tumors of bone

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Table 30.71 Markers for chondroblastoma

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Table 30.72 Inflammatory myofibroblastic tumor (IMT)/inflammatory fibrosarcoma (IF) versus dendritic reticulum cell tumor (DCT)

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Table 30.73 Inflammatory myofibroblastic tumor (IMT)/inflammatory fibrosarcoma (IF) versus sarcomatoid urothelial carcinoma (SUC)

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Table 30.74 Myxoinflammatory fibroblastic sarcoma (MFS) versus Hodgkin’s lymphoma (HL)

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Table 30.75 Fibrosarcoma (CF) versus monomorphic synovial sarcoma (MSS) versus malignant peripheral nerve sheath tumor (MPNST) versus spindle cell carcinoma (SCC) versus spindle cell melanoma (SCM) versus dermatofibrosarcoma protuberans (DFSP) versus spindle cell rhabdomyosarcoma (SCR) versus leiomyosarcoma (LMS) versus spindle cell angiosarcoma (SCA)

Full size table

Table 30.76 Low-grade fibromyxoid sarcoma (LGFS) versus neurofibroma/perineurioma versus desmoid tumors

Full size table

Table 30.77 Juvenile xanthogranuloma (JXG)/reticulohistiocytoma (RC) versus Langerhans cell histiocytosis (LCH)

Full size table

Table 30.78 Dermatofibroma protuberans (DFSP) versus dermatofibroma (DF) versus fibrosarcoma arising in DFSP

Full size table

Table 30.79 Rhabdomyosarcoma (RMS) versus Ewing sarcoma (ES) versus neuroblastoma (NB) versus desmoplastic small round cell tumor (DSRCT) versus synovial sarcoma (SS) versus lymphoma

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Table 30.80 Leiomyoma versus GIST versus schwannoma

Full size table

Table 30.81 Glomus tumors (GT) versus hemangiopericytoma (HPC)/solitary fibrous tumor (SFT) of soft tissue

Full size table

Table 30.82 Kaposiform hemangioendothelioma (KH) versus juvenile capillary hemangioma (CH)

Full size table

Table 30.83 Neurofibroma versus schwannoma versus perineurioma

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Table 30.84 ES/PNET versus lymphoblastic lymphoma (LL)

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Table 30.85 ES/PNET versus neuroblastoma

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Table 30.86 Chordoma versus chondrosarcoma

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Table 30.87 Chordoma versus renal cell carcinoma

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Table 30.88 Langerhans’ cell histiocytosis (LCH) versus Hodgkin lymphoma (HL)

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Table 30.89 Epithelioid sarcoma (ES) versus epithelioid angiosarcoma (EA) versus epithelioid malignant peripheral nerve sheath tumor (EPNST) versus epithelioid leiomyosarcoma (ELMS) versus sclerosing epithelioid fibrosarcoma (SEF) versus epithelioid osteosarcoma (EO)

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Table 30.90 Rhabdomyoma versus granular cell tumor

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Table 30.91 Rhabdomyosarcoma versus leiomyosarcoma

Full size table

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Department of Pathology and Laboratory Medicine, Albany Medical Center, Albany, NY, USA
George Lin
Department of Laboratory Medicine, Geisinger Medical Center, Danville, PA, USA
Shaobo Zhu

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Department of Laboratory Medicine, Geisinger Medical Center, Danville, PA, USA
Fan Lin
Department of Laboratory Medicine, Geisinger Medical Center, Danville, PA, USA
Jeffrey W. Prichard
Department of Laboratory Medicine, Geisinger Medical Center, Danville, PA, USA
Haiyan Liu
Department of Laboratory Medicine, Geisinger Medical Center, Danville, PA, USA
Myra L. Wilkerson

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Lin, G., Zhu, S. (2022). Soft Tissue and Bone Tumors. In: Lin, F., Prichard, J.W., Liu, H., Wilkerson, M.L. (eds) Handbook of Practical Immunohistochemistry. Springer, Cham. https://doi.org/10.1007/978-3-030-83328-2_30

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DOI: https://doi.org/10.1007/978-3-030-83328-2_30
Published: 15 June 2022
Publisher Name: Springer, Cham
Print ISBN: 978-3-030-83327-5
Online ISBN: 978-3-030-83328-2
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