Case 8 -
Syed Z. Ali
The Johns Hopkins Hospital
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35 year-old man with left lower chest wall pain for one year, who has noticed a slowly growing mass
for two months. Radiologically-guided (ultrasound) FNA of the 2.5 cm soft tissue mass (left lateral
lower chest wall).
Smears are hypercellular and comprised of predominantly large irregular tissue fragments with jagged
or feathery outer edges. These fragments seem to be associated with a loose myxomatous stroma which is
scant and identifiable only on closer examination. Higher magnification additionally reveals numerous
discohesive single cells with well-formed fusiform morphology i.e., plump cigar shaped nuclei and long
bipolar cytoplasmic tails. On Papanicolaou stained smears, an elaborate arborizing capillary network is
seen intimately associated with the proliferating mesenchymal cells with stellate shapes, tapering nuclei
and fine wispy cytoplasmic processes. Some tissue fragments also display a loose storiform pattern.
Occasional strands of keloid-like fibrous tissue are also noted.
Histologic and Clinical Follow-up:
The indications for removal i.e., pain and discomfort, and the potential for continued growth were
discussed with the patient. He was very eager to have this removed.
Under general endotracheal anesthesia, the lesion was removed with the surrounding tissue.
Grossly, the specimen consisted of an irregular mass of connective
tissue and skeletal muscle which measured in aggregate 6.0 x 4.0 x
4.5 cm. On cut section, a nodular poorly circumscribed white firm
area was identified which measured 3.5 x 3.0 x 2.5 cm.
Histologic examination showed "NODULAR FASCIITIS. LESION PRESENT AT THE DEEP SPECIMEN MARGIN OF
Last clinical follow-up was in September 2006. He was noted to be doing fairly well.
Nodular fasciitis (NF), also known as "pseudosarcomatous fasci itis" is a benign tumefactive pro
liferation of fibroblasts and myofibroblasts and is a well-known member of the family of pseudotumors.
The lesional cells in NF are reactive rather than neoplastic. It is a self-limited reactive process that
manifests as a readily palpable mass-forming lesion. NF comes to clinical attention as a rapidly
growing, solitary, subcutaneous or superficial soft tis sue mass that often causes alarm for the patient
and the treating physician. NF arises in the upper extremities of adults. The volar surface of the
forearm, followed by the trunk, chest wall, and back are common sites ofoccurrence, but NF has been
reported in many other locations.NF affects the soft tissues at a subcuticular or fascial level but
may extend to the deeper skeletal muscle. NF can be divided into three subtypes based on their
relationship with the fascia: subcutaneous, intramuscular, and fascial. Uncommon clinical and
pathological variants of nodular fasciitis, such as intravascular, cranial, ossifying, and proliferative
fasciitis, have been described. Due to the superficial location, NF is easily amenable to FNA. A
history of trauma might precede NF, but its cause remains unclear. The clinical course is completely
benign, but the high risk of misinterpreting NF as a malignant process owing to its
rapid growth, high cellularity, and high mitotic activity is well-known.
Histologic characteristics of NF are varied and include plump immature fibroblast-like spindle cells,
chronic inflammatory cells, microvascular proliferation, extravasated red blood cells and mucoid matrix
material. In addition to the cytological findings described above in this case, NF are known to have
occasional large ganglion-like cells, background mucoid matrix, foamy cells, cells resembling reapir
phenomenon in exfoliative cytopathology and fragments of atrophic or degenerate skeletal muscle tissue.
NF are easily and often misdiagnosed even by experienced pathologists both on FNA and histopathology
with a reported error rate of as high as 50%. From a cytopathologic perspective, the main differential
diagnosis of NF includes DFSP , low- grade myxofibrosarcoma, and synovial sarcoma. NF tends to have a
characteristic superficial location with a distinct well-circumscribed nodular appearance. The cells in
NF have nuclei that are not hyperchromatic, the lesion does not extend to the skin, mitoses are rare and
when present are not atypical and the amount of collagenized stroma is much smaller than is typically
seen in fibrosarcomas.
NF bears a close resemblance to organizing granulation tissue, and myofibroblastic proliferation may
be initiated by a local injury or local inflammatory process, which supports a reactive proliferation
triggered by trauma. As most cases are surgically excised the natural history of NF is not well
understood. However, local recurrence is rare and those managed conservatively usually resolve
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