Nine smooth muscle tumours, arising at a variety of sites and showing
granular cell change of their cytoplasm, have been studied morphologic
ally and immunohistochemically. The age of the patients ranged from 6
to 78 years (median 42 years); seven patients were female. Two rumours
each arose in the dermis or subcutaneous tissue while the other five
cases were situated in deeper soft tissue. Three of the lesions arose
in the lower limbs, two in the pelvis and one each in the regions of t
he elbow, shoulder, breast and buttock. Follow-up in eight patients wa
s available and revealed local recurrence in three and pulmonary metas
tases in two cases. All cases showed at least focally the light micros
copic features of a smooth muscle tumour and demonstrated moderate to
strong positivity for a-smooth muscle actin. Five were also HHF-35 pos
itive and three were desmin positive. Noteworthy was strong positivity
for the 'melanoma associated' antigen NKI/C3 in all cases. Six cases
stained also weakly positive for NSE, but all were S-100 protein negat
ive. The former is not specific but is the most reliable marker of les
ions showing granular cell change. Granular cytoplasmic change represe
nts simply a cytological phenotype, apparently representing a characte
ristic metabolic alteration, not exclusively associated with Schwann c
ell rumours. Tumours containing granular cells are best classified acc
ording to their line of specific cellular differentiation if possible.