Neuromuscular

TUBULAR AGGREGATES


Tubular aggregates

  Differential Diagnosis

  Pathology
  • Location: Cytoplasm
  • Origin: Sarcoplasmic reticulum
  • Histochemistry: Similar to Cylindrical spirals
    • H & E
      • Irregular, internal regions, slits or lakes
      • May be clear, or contain pink material
    • Gomori trichrome
      • Contain red material
      • Location: Central or sub-sarcolemmal
    • VvG stain: Gray
    • NADH: Stain strongly
    • ATPase: Often in type II > I muscle fibers
    • Sudan: Tubular aggregates contain lipid
    • AMPDA: Stain strongly
    • No reaction
      • Mitochondrial stains
      • Acid & Alkaline phosphatase
  • Protein contents 1
    • DHPR: T-tubules
    • RyR1: Terminal cisternae; Sarcoplasmic reticulum
    • SERCA1
    • : Longitudial sarcoplasmic reticuilum
    • Other: In some patients
  • Ultrastructure (below): 3 types




H & E stain
Muscle fibers
  Pink cytoplasmic areas: May contain nuclei
  Irregular-shaped clear regions

H & E stain

Congo red stain


Gomori trichrome stain


Tubular aggregates
  Stain red on Gomori trichrome

Gomori trichrome stain


Gomori trichrome stain
Tubular aggregates: May have elongated or rounded shapes

Gomori trichrome stain

Gomori trichrome stain


VvG stain

Tubular aggregates
  Stain gray-black on VvG


NADH-TR reductase (NADH) stain



Tubular aggregates:
  Stain dark on NADH

NADH stain


AMPDA stain
Tubular aggregates: Contain AMPDA

AMPDA stain


ATPase pH 9.4 stain
Tubular aggregates (Pale regions): Are often more common in type 2 muscle fibers

ATPase pH 4.3 stain


Sudan black stain
Tubular aggregates: Stain for lipid on Sudan black

Sudan black stain

Tubular aggregates
  May have mild or patchy staining for Acid Phosphatase

Acid Phosphatase stain

Tubular aggregates: Ultrastructure; 3 types


From T Mozaffar

Return to Myopathies with tubular aggregates

References
1. J Neuropathol Exp Neurol 2016;75:1171-1178


12/14/2016