Comments are encouraged!!!!
Clinical
History
1. Gender of patient: __male
___female
2. Age at presentation: _
_ years _ _ months
3. Age at biopsy: _ _ years _ _ months
4. Symptoms at presentation (check all that
apply):
Weakness
Hypotonia
Muscle pain
Cardiac
disease
Central
nervous system disease
Respiratory
difficulties
Contractures
Failure to
thrive
Others (see
item 8)
5. Elevated
creatine kinase: Yes No Unknown _______
Patient Value _________(Normal Range)
6. Familial
Inheritance:____None ___Autosomal Recessive ____Autosomal Dominant ____X-linked
7. EMG
Findings:
____Not known ____Myopathic ____Neuropathic
8. Other
symptoms, signs, and lab data: ____________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________
Muscle Biopsy Tissue
Information
1.
Name of Muscle:___________________________________________________________________
2.
Size of tissue collected*: ______
X ______ X
______ cm
3.
Date of tissue collection*: __ __ / __ __ / __ __ __ __
m m
d d y
y y y
4.
Biopsy method: Open Needle
5.
Freezing or Fixation Used*? Frozen: Amount: _____ mg Not known
Formalin-fixed: Amount: _____ mg Not known
Paraffin-embedded: Amount: _____ mg Not known
Epon-embedded: Amount: _____ mg Not known
Histological
Findings in Muscle Biopsy or Autopsy specimens
1. Which
standard histochemical stains were used*? (choose all that apply)
H and E Gomori
trichrome NADH COX SDH
COX/SDH PAS Oil Red O ATPase 4.3 ATPase 4.6
ATPase 9.4 Other,
specify: __________________________________________________________________________________________________________________________________________________________________________________
2. Which
of the following diagnostic abnormalities were noted on histochemical stains
(choose all that apply)*?
Fatty replacement ___absent ___mild ___moderate ___severe
Endomysial fibrosis ___absent ___mild ___moderate ___severe
Myofiber degeneration ___absent ___mild ___moderate ___severe
Necrosis ___absent ___mild ___moderate ___severe
Myophagocytosis ___absent ___present in ____ fibers
Myofiber regeneration
(Basophilic fibers) ____absent ___present in _____ fibers
Abnormalities
of fiber type ____absent _____present
Specify*: Type 1
predominance ______ % Type 1 fibers
Type 2
predominance ______% Type 2 fibers
Fiber type
grouping (of both fiber types)
Hypertrophic fibers ____absent _____present in _____ fibers
Atrophy/Hypotrophy ____absent _____present
Specify: All fibers
within the specimen
Subsets of
fibers, leading to excessive variation in fiber size
Specify (choose all that
apply): Single
fibers Groups of
fibers
Type 1
fibers only Type 2
fibers only
Perifascicular distribution
Atrophic/hypotrophic fiber shape
Angulated Round
Myopathy-associated
pathological structures, specify:
Central nuclei _____absent _____present
Specify estimated % of fibers
(include eccentric nuclei): _____
Internal nuclei _____absent _____present
Specify estimated % of fibers
(if not quantified above): _____
Inclusion bodies ____absent ____present in _____ fibers
Rimmed vacuoles ____absent ____present in _____ fibers
Nemaline rods ____absent ____present
Specify: Restricted
to one fiber type, specify which: _____
Nuclear
rods present
Ragged red fibers ____absent ____present in _____fibers
COX- negative fibers Estimated number ______
Strongly SDH-reactive blood
vessels (SSV’s) _____absent _____present
Central cores ____absent ____present in _____ fibers
Minicores ____absent ____present
in ____ fibers
Core-like lesions ____absent ____present in ____ fibers
Targetoid fibers ____absent ____present in ____ % of fibers
Marked hypotrophy of type 1 fibers ____absent ____present
Inflammation ___absent ___mild ___moderate ___severe
Specify:
Distribution
Perivascular
Evidence of
vascular damage Thrombi
identified in blood vessels
Focal
Diffuse
Endomysial
Perimysial
Involving
fascia
Associated
with myofiber damage
Associated
with non-necrotic myofiber
Granulomas
Necrotizing
Non-necrotizing
Giant cells present Foreign
material present
Inflammatory cells identified
Specify (choose all that
apply):
Lymphocytes
Neutrophils
Macrophages
Eosinophils
(as a prominent component)
Microorganisms identified, specify which:
_________________________________________
Abnormal
storage material
Specify:
Excessive glycogen ____absent ____mild ____severe
Excessive intracellular
lipid ____absent ____mild ____severe
Additional
observations
____________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________________
3. Which
immunohistochemical stains were used? (choose all that apply)
Myosin
immunohistochemistry (for fast and slow fibers)
Dystrophin
panel
Specify:
Dystrophin (DYS1) ____absent ____reduced ____normal
Dystrophin (DYS2) ____absent ____reduced ____normal
Dystrophin (DYS3) ____absent ____reduced ____normal
Dystrophin (BMD Hotspot) ____absent ____reduced ____normal
Spectrin ____absent ____reduced ____normal
Utrophin ____absent ____normal ____increased
Other
stains for limb-girdle or congenital muscular dystrophy
Specify:
Laminin a2/Merosin ____absent ____reduced ____normal
Alpha dystroglycan (VIA) ____absent ____reduced ____normal
Alpha dystroglycan (IIH) ____absent ____reduced ____normal
Beta dystroglycan ____absent ____reduced ____normal
Alpha sarcoglycan ____absent ____reduced ____normal
Beta sarcoglycan ____absent ____reduced ____normal
Delta sarcoglycan ____absent ____reduced ____normal
Gamma sarcoglycan ____absent ____reduced ____normal
Dysferlin ____absent ____reduced ____normal
Emerin ____absent ____reduced ____normal
Collagen VI ____absent ____reduced ____normal
Caveolin 3 ____absent ____reduced ____normal
Desmin ____absent ____reduced ____normal
Integrin a7 ____absent ____reduced ____normal
nNOS ____absent ____reduced ____normal
Inflammatory myopathy panel
CD4 ____absent ____present in ___ % of lymphocytes
CD8 ____absent ____present in ___ % of lymphocytes
CD20 ____absent ____present in ___ % of lymphocytes
CD45 ____absent ____present in ____% of mononuclear cells
CD68 ____absent ____present in ____% of mononuclear cells
C5b-9 ____absent ____present on endomysial capillary walls
Major Histocompatability
Complex ____absent ____sarcolemmal ____diffuse
4. Additional immunohistochemical/immunofluorescence
assays performed:
__________________________________________________________________________________
5. Other
abnormalities noted on immunohistochemistry: __________________________________
Epon-Embedded
Tissue/Electron Microscopy (Muscle Biopsy/Autopsy Specimens)
1. Abnormalities
seen on: Light
microscopy (Toluidine blue staining) Electron
microscopy
Both –
Light microscopy and Electron microscopy
2. Abnormalities
noted in: Contractile
apparatus
Sarcotubular organization
Mitochondria, specify (choose all that apply):
Abnormal
shape
Abnormal
numbers
Abnormal
location
Abnormal
architecture
3. Describe
any pathological inclusions noted: N/A
_________________________________________________________________________________________
_________________________________________________________________________________________
4. Describe
any abnormal storage material identified: N/A
_________________________________________________________________________________________
_________________________________________________________________________________________
Interpretation:
Comment:
6 comments:
Comprehensive, but way over the top for a clinical service. If I had to do this for every muscle/nerve specimen I have, I'd get no other work done. This is best used as part of a research protocol.
JD: The same comment you made has been coming up regarding the pituitary checklist. Perhaps these checklists need to be split into two parts: The first section for diagnostic purposes only and the second part for research purposes. No doubt over the years some of the research data will migrate to the diagnostic section.
I applaud efforts to develop checklists (I love Atul Gawande's Checklist Manifesto), but share similar reservations about complex standards that might lack immediate clinical relevance or reproducibility.
Part of the problem with big checklists is wrestling with data entry and report generation. "Free texting" complex reports is a recipe for errors and is way too time-consuming. Our practice (Hospital Pathology Associates, MN)tries to get around this by using FileMaker Pro solutions to streamline tumor staging, for example. Our FMP solutions generate reports that we paste into our LIS (alas, we don't have the IT support to work out a direct connection). It cuts down on the hassle factor of big protocols and reduces errors.
Very thorough. I especially like the first part about clinical information. I'm going to incorporate that into the form we have extramural clinicians fill out when shipping us consult cases. They'll probably still ignore 90% of the fields, but at least we're trying.
Would add CD45, CD56, vimentin, and maybe slow/fast myosin.
And how 'bout adding CD138 and p62?
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