Difference between revisions of "Granulocytic sarcoma"

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cases can occasionally precede full blown AML
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'''Granulocytic sarcoma''' is an uncommon [[malignant]] [[soft tissue lesion]] that is really represents a hematologic malignancy; it is a soft tissue manifestation of [[acute myeloid leukemia]]. It is ''not'' a [[sarcoma]].
{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Chloroma_-_very_high_mag.jpg
| Width      =
| Caption    = Chloroma. [[H&E stain]].
| Synonyms  = extramedullary leukemia, myeloid sarcoma and chloroma, myeloblastoma, chloromyeloma, chloromyelosarcoma, granulocytic leukosarcoma, myelosarcoma
| Micro      = atypical small blue cells ~2x resting lymphocyte, infiltrative
| Subtypes  =
| LMDDx      = [[small round cell tumours]]
| Stains    =
| IHC        = CD117 +ve, CD43 +ve, CD34 +ve/-ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = [[soft tissue lesion]]
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    = other [[soft tissue lesions]]
| Tx        = see ''[[acute myeloid leukemia]]''
}}
'''Granulocytic sarcoma''' is an uncommon [[malignant]] [[soft tissue lesion]] that really represents a hematologic malignancy; it is a soft tissue manifestation of [[acute myeloid leukemia]]. It is ''not'' a [[sarcoma]]. In a small number of cases, granulocytic sarcoma may precede systemic disease and may show a aleukaemic picture, i.e. there may not be significant numbers of blasts circulating in the blood.


Numerous other terms refer to this including '''extramedullary leukemia''',<ref name=pmid21795742>{{Cite journal  | last1 = Bakst | first1 = RL. | last2 = Tallman | first2 = MS. | last3 = Douer | first3 = D. | last4 = Yahalom | first4 = J. | title = How I treat extramedullary acute myeloid leukemia. | journal = Blood | volume = 118 | issue = 14 | pages = 3785-93 | month = Oct | year = 2011 | doi = 10.1182/blood-2011-04-347229 | PMID = 21795742 }}</ref> '''myeloid sarcoma''' and '''chloroma'''.  
Numerous other terms refer to this including '''extramedullary leukemia''',<ref name=pmid21795742>{{Cite journal  | last1 = Bakst | first1 = RL. | last2 = Tallman | first2 = MS. | last3 = Douer | first3 = D. | last4 = Yahalom | first4 = J. | title = How I treat extramedullary acute myeloid leukemia. | journal = Blood | volume = 118 | issue = 14 | pages = 3785-93 | month = Oct | year = 2011 | doi = 10.1182/blood-2011-04-347229 | PMID = 21795742 }}</ref> '''myeloid sarcoma''' and '''chloroma'''.  
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==General==
==General==
*Soft tissue manifestation of [[acute myeloid leukemia]].<ref name=pmid21556238/>
*Soft tissue manifestation of [[acute myeloid leukemia]].<ref name=pmid21556238/>
*WBC elevated, low or normal range.<ref name=pmid25678833>{{Cite journal  | last1 = Arthur | first1 = C. | last2 = Cermak | first2 = J. | last3 = Delaunay | first3 = J. | last4 = Mayer | first4 = J. | last5 = Mazur | first5 = G. | last6 = Thomas | first6 = X. | last7 = Wierzbowska | first7 = A. | last8 = Jones | first8 = MM. | last9 = Berrak | first9 = E. | title = Post hoc analysis of the relationship between baseline white blood cell count and survival outcome in a randomized Phase III trial of decitabine in older patients with newly diagnosed acute myeloid leukemia. | journal = J Blood Med | volume = 6 | issue =  | pages = 25-9 | month =  | year = 2015 | doi = 10.2147/JBM.S64067 | PMID = 25678833 }}</ref>


==Microscopic==
==Microscopic==
Features:
Features:
*Cluster of atypical small blue cells in [[soft tissue lesions|soft tissue]].
*Cluster of atypical small blue cells in [[soft tissue lesions|soft tissue]] with scant cytoplasm.


Note:
DDx:
*May mimic [[small cell carcinoma]], large cell lymphomas ([[DLBCL]], [[ALCL]]), [[small round cell tumour]]s.
*[[Small cell carcinoma]]
*Large cell lymphomas ([[DLBCL]], [[ALCL]]).
*Other [[small round cell tumour]]s.


===Images===
===Images===
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Image:Chloroma_-_high_mag.jpg | Chloroma - high mag. (WC)
Image:Chloroma_-_high_mag.jpg | Chloroma - high mag. (WC)
Image:Chloroma_-_very_high_mag.jpg | Chloroma - very high mag. (WC)
Image:Chloroma_-_very_high_mag.jpg | Chloroma - very high mag. (WC)
Image:Myeloid sarcoma within a lymph node x40 magnification.jpg | Myeloid sarcoma within a lymph node (WC)
</gallery>
</gallery>
www:
www:
*[http://path.upmc.edu/cases/case306/micro.html Granulocytic sarcoma - several crappy images (upmc.edu)].
*[http://path.upmc.edu/cases/case306/micro.html Granulocytic sarcoma - several crappy images (upmc.edu)].
*[http://path.upmc.edu/cases/case379.html Myeloid sarcoma - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case379.html Myeloid sarcoma - several images (upmc.edu)].
==IHC==
Features:<ref name=pmid24969631>{{Cite journal  | last1 = Seifert | first1 = RP. | last2 = Bulkeley | first2 = W. | last3 = Zhang | first3 = L. | last4 = Menes | first4 = M. | last5 = Bui | first5 = MM. | title = A practical approach to diagnose soft tissue myeloid sarcoma preceding or coinciding with acute myeloid leukemia. | journal = Ann Diagn Pathol | volume = 18 | issue = 4 | pages = 253-60 | month = Aug | year = 2014 | doi = 10.1016/j.anndiagpath.2014.06.001 | PMID = 24969631 }}</ref>
Markers of immaturity:
*CD34 +ve/-ve (5 of 9 cases).
*CD117 +ve (9 of 9 cases).
*TdT
Myeloid markers:
*CD43 +ve (7 of 7 cases) - sensitive, but not specific
*Myeloperoxidase +ve (8 of 10 cases).
*CD11c (myelomonocytic marker)
*CD13 (granulopoietic marker)
*CD33 (granulopoietic marker, specific but less sensitive)
CD34, CD117 and myeloperoxidase are more commonly positive in cases showing granulopoietic differentiation, but can be negative in cases with a myelomonocytic or monocytic differentiation, where CD68, CD163 and lysozyme may be helpful.<ref name=pmid23530613>{{cite journal |vauthors=Zhou J, Bell D, Medeiros LJ |title=Myeloid sarcoma of the head and neck region |journal=Arch Pathol Lab Med |volume=137 |issue=11 |pages=1560–8 |date=November 2013 |pmid=23530613 |doi=10.5858/arpa.2012-0537-OA |url=}}</ref>


==Sign out==
==Sign out==
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==See also==
==See also==
*[[Soft tissue lesions]].
*[[Soft tissue lesions]].
*[[Intravascular lymphoma]].


==References==
==References==
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