Soft tissue
Skeletal muscle
Rhabdomyosarcoma
Spindle cell / sclerosing rhabdomyosarcoma
Author: Erdener Özer, M.D., Ph.D.
Editorial Board Member: Michael R. Clay, M.D.
Editor-in-Chief: Debra L. Zynger, M.D.
Last author update: 1 May 2018
Last staff update: 2 November 2022 (update in progress)
Copyright: 2002-2023, PathologyOutlines.com, Inc.
PubMed Search: Sclerosing rhabdomyosarcoma
Table of Contents
Definition / general | Essential features | Terminology | Clinical features | Radiology images | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Cytology images | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Differential diagnosis | Additional references | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Özer E. Spindle cell / sclerosing rhabdomyosarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuesclerosingrhabdo.html. Accessed January 30th, 2023.
Definition / general
- Spindle cell / sclerosing rhabdomyosarcoma (RMS) is a rare variant
- Spindle cell pattern was proposed in 1992 by Cavazzana et al. and the sclerosing pattern was described in 2000 by Mentzel and Katenkamp (Am J Surg Pathol 1992;16:229, Virchows Arch 2000;436:305)
- Predilection for the head and neck / extremities
- 5 - 10% of RMS (IARC: WHO Classification of Tumours of Soft Tissue and Bone, 4th edition, 2013, Arch Pathol Lab Med 2015;139:1281)
Essential features
- Variant of rhabdomyosarcoma with dense hyalinizing collagenous matrix with rounded or spindle shaped tumor cells arranged in small nests present in at least 80% of the tumor (Am J Surg Pathol 2002;26:1175, Am J Clin Pathol 2008;129:410)
Terminology
- Spindle cell / sclerosing are considered the same diagnostic variant of rhabdomyosarcoma
- Previously categorized as a part of embryonal rhabdomyosarcoma
Clinical features
- Sclerosing: more common in adults in the extremities and head and neck region (Am J Surg Pathol 2002;26:1175)
- Spindle cell: most common in the paratestis, followed by head and neck
- More common in the pediatric population in the extremities (Arch Pathol Lab Med 2015;139:1281)
- Males > females
Radiology images
Images hosted on other servers:
Large masticator space mass in neck
Cranial MRI
CT scan
MRI
Prognostic factors
- In adults, recurrence and metastasis rate of 40 - 50% (Arch Pathol Lab Med 2015;139:1281)
- In pediatric population, similar behavior to embryonal variant except parameningeal spindle cell is more aggressive (Arch Pathol Lab Med 2015;139:1281)
- Prognosis of tumors in the head and neck is worse than those located in the extremities
Case reports
- 24 year old man with skull tumor (Int J Clin Exp Pathol 2015;8:2205)
- 26 year old woman with a large chest wall tumor (Ann Thorac Cardiovasc Surg 2014;20:642)
- 32 year old man with swelling of right masseter (Case of the Week #457)
- 35 year old man presenting with knee tumor (Indian J Pathol Microbiol 2015;58:335)
- 36 year old Latino man with parotid gland tumor (Head Neck Pathol 2015;9:147)
- 50 year old man with oral rhabdomyosarcoma (Indian J Otolaryngol Head Neck Surg 2016;68:384)
- 55 year old woman with a right thigh mass (Diagn Cytopathol 2010;38:761)
- 67 year old Japanese woman with tumor in the masticator space (Acta Radiol Open 2017;6:2058460117727316)
Treatment
- No consensus for optimal treatment
- Mainstay therapeutic method should be similar to most soft tissue tumors including surgery and adjuvant chemotherapy or radiotherapy can be added (Int J Clin Exp Pathol 2015;8:13814)
Clinical images
Images hosted on other servers:
Cystic mass 10 × 10 × 8 cm
Gross description
- Poorly circumscribed, fleshy, expanding masses
Microscopic (histologic) description
- Neoplastic cells are arranged in lobules, small nests, microalveoli and single file arrays in an abundant hyalinized, eosinophilic to basophilic matrix that closely resembles primitive osteoid or chondroid material
- Mitotic rate is very high (> 20 mitotic figures / 20 high power fields)
- Spindle cells or small primitive-like cells embedded in abundant in collagen stroma
- Scattered rhabdomyoblasts with eccentric nuclei and significant ample eosinophilic cytoplasm
- In some cases tumor cells focally display a cribriform pattern closely mimicking adenoid cystic carcinoma
Microscopic (histologic) images
Contributed by Dr. Saroona Haroon, Case #457
x10
x20
x20
x40
x40
CD34
Desmin
S100
MSA
MyoD1
Myogenin
Myoglobin
Images hosted on other servers:
Hyalinizing collagenous matrix
Neoplastic cells arranged into microalveoli
Neoplastic cells arranged into lobules
Hyalinized matrix resembles osteoid
IHC positivity to desmin, myogenin, SMA, MyoD1
Spindle cell and sclerosing features
Uniformly distributed tumor cells
H&E, desmin, myogenin
Cytology images
Images hosted on other servers:
Fine needle aspiration
Positive stains
Negative stains
- Cytokeratin, S100, CD34 and CD31
Electron microscopy description
- Irregularly distributed disorganized filament in richly collagenized matrix
- No Z bands
Molecular / cytogenetics description
- Should be PAX fusion negative
- Recurrent MyoD1 mutations (Genes Chromosomes Cancer 2014;53:779)
- Specific MDM2 / HMGA2 amplification at 12q13 - 15 (Hum Pathol 2009;40:1347)
- Loss of 10q22, loss of chromosome Y and gain of 18 (Virchows Arch 2006;449:572)
- Pattern of numerical changes similar to embryonal rhabdomyosarcoma in one case (Virchows Arch 2005;446:64)
- In an adult case: gain of chromosome 11 and loss of chromosome 22 (Virchows Arch 2005;446:64)
- Cytogenetic findings in 2 pediatric cases suggest a link with embryonal rhabdomyosarcoma (Int J Surg Pathol 2006;14:193)
Differential diagnosis
- Alveolar rhabdomyosarcoma (RMS): different morphology; has PAX3 and PAX7-FKHR fusion transcripts (Pediatr Dev Pathol 2004;7:583) and myogenin is generally seen as weak, focal and nuclear
- Angiosarcoma: lacks characteristic hyalinizing matrix of sclerosing RMS, is negative for desmin, myogenin and MyoD1, but is CD31 and CD34 positive (Head Neck Pathol 2015;9:147)
- Extraskeletal myxoid chondrosarcoma: typically forms a well circumscribed, multilobulated architecture separated by incomplete fibrous septa with round or slightly elongated cells of uniform shape and size usually arranged in short anastomosing strands or cords in myxoid matrix
- Mesenchymal chondrosarcoma: characterized by distinct undifferentiated tumor cells admixed with well differentiated cartilagenous components
- Metastatic carcinoma: positive expression of cytokeratin is seen in metastatic carcinoma, whereas desmin and MyoD1 are negative
- Osteosarcoma: has neoplastic osteoid, matrix calcification, osteoclasts
- Parachordoma: typically lobulated and contains nests of vacuolated cells deposited in a myxoid matrix, resembling the physaliphorous cells of chordoma; expresses S100 protein and cytokeratin
- Sclerosing epithelioid fibrosarcoma: composed of epithelioid cells arranged in nests and cords and deposited in a densely hyalinized collagenous matrix; however, in almost all cases the tumor also shows foci of spindle shaped sarcoma similar to conventional fibrosarcoma and exhibits EMA or MUC4 positivity (Head Neck Pathol 2015;9:147)
- Also synovial sarcoma, leiomyosarcoma, spindle cell carcinoma, spindle cell melanoma, fibrosarcoma, malignant peripheral nerve sheath tumor, and malignant triton tumor
Additional references
Board review style question #1
- Which of the following antibodies is strongly diffuse positive in sclerosing rhabdomyosarcoma?
- Actin
- CD99
- Desmin
- MyoD1
- Myogenin
Board review style answer #1
Board review style question #2
Which of the following is associated with sclerosing rhabdomyosarcoma?
A. PAX7-FOXO1A fusion
B. Polypoid growth with a cambium layer
C. Arises only in adults over 45
D. Pseudovascular growth pattern
A. PAX7-FOXO1A fusion
B. Polypoid growth with a cambium layer
C. Arises only in adults over 45
D. Pseudovascular growth pattern
Board review style answer #2
D. Sclerosing rhabdomyosarcomas often display a pseudovascular growth pattern.
PAX7-FOXO1A fusion is associated with the alveolar subtype; polypoid growth with a
cambium layer is seen in the botryoid type; and the pleomorphic subtype is seen in
older patients.
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Reference: Spindle cell / sclerosing rhabdomyosarcoma
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Reference: Spindle cell / sclerosing rhabdomyosarcoma
