Difference between revisions of "Rhabdomyoma"

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Rhabdomyoma -- high mag.jpg
| Width      =
| Caption    = Rhabdomyoma. [[H&E stain]]. (WC)
| Synonyms  =
| Micro      = spider cells (large polygonal cells (~10-20x RBC diameter), abundant cytoplasm with clearing)
| Subtypes  = fetal rhabdomyoma, adult rhabdomyoma
| LMDDx      = [[granular cell tumour]], [[hibernoma]], [[oncocytoma]]
| Stains    =
| IHC        = desmin +ve, actin +ve, myoglobin +ve, S-100 -ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Staging    =
| Site      = [[heart]] (see ''[[cardiac tumours]]), [[head and neck pathology|head and neck]]
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = benign
| Other      =
| ClinDDx    =
| Tx        =
}}
'''Rhabdomyoma''' a benign muscle tumour.  Often seen in the context of [[tuberous sclerosis]].
'''Rhabdomyoma''' a benign muscle tumour.  Often seen in the context of [[tuberous sclerosis]].


==General==
==General==
*May be seen in the context of [[tuberous sclerosis]].
*May be seen in the context of [[tuberous sclerosis]].
*Rare benign mesenchymal tumour - may be seen in the [[head and neck pathology|head and neck]].<ref name=pmid16133368/>
*Can cause death if in the [[cardiac tumours|heart]].<ref name=pmid23206573 >{{Cite journal  | last1 = Neri | first1 = M. | last2 = Di Donato | first2 = S. | last3 = Maglietta | first3 = R. | last4 = Pomara | first4 = C. | last5 = Riezzo | first5 = I. | last6 = Turillazzi | first6 = E. | last7 = Fineschi | first7 = V. | title = Sudden death as presenting symptom caused by cardiac primary multicentric left ventricle rhabdomyoma, in an 11-month-old baby. An immunohistochemical study. | journal = Diagn Pathol | volume = 7 | issue =  | pages = 169 | month = Dec | year = 2012 | doi = 10.1186/1746-1596-7-169 | PMID = 23206573 }}</ref>


==Gross==
==Gross==
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**Large polygonal cells (~10-20x RBC diameter):
**Large polygonal cells (~10-20x RBC diameter):
***Abundant cytoplasm filled with glycogen.
***Abundant cytoplasm filled with glycogen.
Note:
*Fetal rhabdomyomas may have [[pseudoepitheliomatous hyperplasia]].<ref name=pmid16133368/>


DDx:
DDx:
*[[Hibernoma]].
*[[Granular cell tumour]].
*[[Oncocytoma]].
*[[Lipoma]].
*[[Lipoma]].
*[[Fat necrosis]].
*[[Fat necrosis]].
*[[Hiberoma]].
*[[Granular cell tumour]].


===Images===
===Images===
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==IHC==
==IHC==
Features:<ref name=pmid16133368 >{{Cite journal  | last1 = Hansen | first1 = T. | last2 = Katenkamp | first2 = D. | title = Rhabdomyoma of the head and neck: morphology and differential diagnosis. | journal = Virchows Arch | volume = 447 | issue = 5 | pages = 849-54 | month = Nov | year = 2005 | doi = 10.1007/s00428-005-0038-8 | PMID = 16133368 }}</ref>
Features:<ref name=pmid16133368 >{{Cite journal  | last1 = Hansen | first1 = T. | last2 = Katenkamp | first2 = D. | title = Rhabdomyoma of the head and neck: morphology and differential diagnosis. | journal = Virchows Arch | volume = 447 | issue = 5 | pages = 849-54 | month = Nov | year = 2005 | doi = 10.1007/s00428-005-0038-8 | PMID = 16133368 }}</ref>
*Desmin +ve.
*Desmin +ve (21 of 21 adult cases<ref name=pmid8505039>{{Cite journal  | last1 = Kapadia | first1 = SB. | last2 = Meis | first2 = JM. | last3 = Frisman | first3 = DM. | last4 = Ellis | first4 = GL. | last5 = Heffner | first5 = DK. | last6 = Hyams | first6 = VJ. | title = Adult rhabdomyoma of the head and neck: a clinicopathologic and immunophenotypic study. | journal = Hum Pathol | volume = 24 | issue = 6 | pages = 608-17 | month = Jun | year = 1993 | doi =  | PMID = 8505039 }}</ref>).
*Myogoblin +ve.
*Myogoblin +ve.
*Actin +ve.
*Actin +ve (21 of 21 adult cases<ref name=pmid8505039/>).
*Vimentin -ve/+ve.
*S-100 -ve.
*S-100 -ve.
**Positive in granular cell tumour and hiberoma.
**Positive in granular cell tumour and hiberoma.
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==See also==
==See also==
*[[Cardiac tumours]].
*[[Cardiac tumours]].
*[[Head and neck pathology]].


==References==
==References==
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[[Category:Soft tissue lesions]]
[[Category:Soft tissue lesions]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:Head and neck pathology]]

Revision as of 15:54, 4 September 2017

Rhabdomyoma
Diagnosis in short

Rhabdomyoma. H&E stain. (WC)
LM spider cells (large polygonal cells (~10-20x RBC diameter), abundant cytoplasm with clearing)
Subtypes fetal rhabdomyoma, adult rhabdomyoma
LM DDx granular cell tumour, hibernoma, oncocytoma
IHC desmin +ve, actin +ve, myoglobin +ve, S-100 -ve
Site heart (see cardiac tumours), head and neck
Prevalence rare
Prognosis benign

Rhabdomyoma a benign muscle tumour. Often seen in the context of tuberous sclerosis.

General

Gross

  • Solid, white/tan colour.

Image:

Microscopic

Features - cardiac:[3]

  • Spider cells:
    • Large polygonal cells (~10-20x RBC diameter):
      • Abundant cytoplasm filled with glycogen.

Note:

DDx:

Images

  • Rhabdomyoma - low mag. (WC)

  • Rhabdomyoma - intermed. mag. (WC)

  • Rhabdomyoma - high mag. (WC)

  • Rhabdomyoma - very high mag. (WC)

  • Rhabdomyoma - desmin - intermed. mag. (WC)

  • Rhabdomyoma - desmin - high mag. (WC)

www

IHC

Features:[1]

  • Desmin +ve (21 of 21 adult cases[4]).
  • Myogoblin +ve.
  • Actin +ve (21 of 21 adult cases[4]).
  • Vimentin -ve/+ve.
  • S-100 -ve.
    • Positive in granular cell tumour and hiberoma.

See also

References

  1. ↑ 1.0 1.1 1.2 Hansen, T.; Katenkamp, D. (Nov 2005). "Rhabdomyoma of the head and neck: morphology and differential diagnosis.". Virchows Arch 447 (5): 849-54. doi:10.1007/s00428-005-0038-8. PMID 16133368.
  2. ↑ Neri, M.; Di Donato, S.; Maglietta, R.; Pomara, C.; Riezzo, I.; Turillazzi, E.; Fineschi, V. (Dec 2012). "Sudden death as presenting symptom caused by cardiac primary multicentric left ventricle rhabdomyoma, in an 11-month-old baby. An immunohistochemical study.". Diagn Pathol 7: 169. doi:10.1186/1746-1596-7-169. PMID 23206573.
  3. ↑ URL: http://www.brown.edu/Courses/Digital_Path/systemic_path/cardio/rhabdomyoma.html. Accessed on: 19 October 2011.
  4. ↑ 4.0 4.1 Kapadia, SB.; Meis, JM.; Frisman, DM.; Ellis, GL.; Heffner, DK.; Hyams, VJ. (Jun 1993). "Adult rhabdomyoma of the head and neck: a clinicopathologic and immunophenotypic study.". Hum Pathol 24 (6): 608-17. PMID 8505039.
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