Case 10

Case Presentation

A 38 year old male complains of

buttock pain….
The Challenge of Spindle Cell
Lesions of Bone

Andrew E Rosenberg, MD
Department of Pathology
University of Miami
Miami, Florida
Arosenberg@miami.edu pathotim
2018
 Spindle Cell Lesions of Bone
Phenotype of Cell Disease Process
Fibroblasts/myofibroblasts Reactive
Smooth muscle fracture callus
Schwann cells Benign neoplasm
Skeletal muscle cells Primary malignant neoplasm
Endothelial cells Metastasis
Myoepithelial cells sarcomatoid carcinoma
Carcinoma cells spindle cell melanoma
Melanoma cells
Lymphoma cells
 Fibrous Tumors of Bone
Benign Malignant
Fibrous cortical defect Fibrosarcoma (MFH)
Non-ossifying fibroma Myofibrosarcoma
Myofibroma/ Solitary fibrous tumor
myofibromatosis
Fibrous histiocytoma Fibroblastic
Desmoplastic fibroma osteosarcoma
Solitary fibrous tumor Dedifferentiated
Reactive chondrosarcoma
Non-union fracture
Periosteal desmoid
 Fibrous Tumors of Bone
Immunohistochemistry – non-specific
Vimentin
Smooth muscle actin
Desmin
CD68
keratin
Genetic aberrations
No specific changes in most
Exceptions – myofibroma, infantile fibrosarcoma,
solitary fibrous tumor, myoepithelioma
A 15 year old boy complains of
knee pain…
 Fibrous Cortical Defect
Non-Ossifying Fibroma

Definition: Benign, proliferation of fibroblasts admixed

with mono/multinucleated histiocytes
intracortical – fibrous cortical defect
medullary cavity – non-ossifying fibroma
Synonyms: Metaphyseal fibrous defect, benign
fibrous histiocytoma
Incidence: 54% of boys; 22% of girls
Age: 1st – 2nd decades of life
 Fibrous Cortical Defect
Non-Ossifying Fibroma
Gender: M:F = 2:1
Location: Metaphysis of long bones (90% lower
extremity)
distal femur > proximal tibia > distal tibia
Multiplicity: Solitary, multiple (Neurofibromatosis
type 1, Jaffe Campanacci syndrome – multiple
non-ossifying fibromas and café-au-lait
macules)
Clinical features: Asymptomatic – incidental finding,
pain, pathologic fracture
Fibrous Cortical Defect
Non-Ossifying Fibroma
Non-Ossifying Fibroma
Multiple Non-Ossifying Fibromas
Neurofibromatosis type 1
Jaffe Campanacci Syndrome
Fibrous cortical Defect
Non-Ossifying Fibroma
Fibrous Cortical Defect
Non-Ossifying Fibroma - Healing
 Fibrous Cortical Defect
Non-Ossifying Fibroma
Treatment
Observation, curettage, segmental
resection
Prognosis
Excellent – most undergo spontaneous
resolution, surgerized cases rarely
recur
 Myofibroma - Myofibromatosis

Age: Present at birth or within first several years

of life
Centricity: Solitary or multiple (bone and
extraskeletal sites) 3:1
Gender: M>F
Genetics: Activating mutations in PDGFRB;
somatic and germline – differing mutations
Location: Extremities > head and neck > trunk
Presentation: Pain, rapidly growing, pathologic
fracture
Myofibroma - Myofibromatosis
Myofibroma - Myofibromatosis
Myofibroma - Myofibromatosis
SMA
 Myofibroma - Myofibromatosis

Treatment
Observation, curettage, segmental
resection
Prognosis
Excellent – infrequently recur,
spontaneous resolution
 Desmoplastic Fibroma
Definition: Benign, locally aggressive, fibroblastic
neoplasm, analogous - different from
fibromatosis
Age: wide range – children to elderly; peak incidence
2nd -3rd decade of life; 75% of patients are <30
years old
Gender: M=F
Location: Metaphysis of long bones, pelvis, jaw
Clinical features: Pain, pathologic fracture
Desmoplastic Fibroma
Desmoplastic Fibroma
Desmoplastic Fibroma
 Desmoplastic Fibroma
Differential Diagnosis
Non-ossifying fibroma
Chondromyxoid fibroma
Myoepithelioma
Periosteal desmoid
Well differentiated fibrosarcoma
Well differentiated osteosarcoma
 Periosteal Desmoid - Avulsive
Cortical Irregularity
Definition: non-neoplastic reactive fibroblastic
proliferation resulting from avulsion of adductor
magnus and medial gastrocnemius insertions
into the posteromedial metaphysis of the distal
femur
Age: 10-15 years
Gender: M>F
Clinical features: Incidental finding, pain, swelling
Location: posteromedial aspect of metaphysis of
distal femur; 1/3 are bilateral
Periosteal Desmoid
 Periosteal Desmoid
Treatment
Observation
Medical therapy – symptom relief
Prognosis
Excellent – heals – no recurrence
 Fibrosarcoma
Definition: Primary malignant tumor of bone that
demonstrates fibroblastic and myofibroblastic
differentiation
Synonyms: Malignant fibrous histiocytoma
undifferentiated pleomorphic sarcoma
Gender: M:F = 1:1
Age: Middle age adults and elderly; > 50years old
Prexisting Conditions: Radiation, infarcts,
Hardcastle disease
Location: Long bones (femur and tibia) and pelvis
Fibrosarcoma
Fibrosarcoma
Fibrosarcoma
Keratin
 Fibrosarcoma
Treatment
Staging
Wide excision – may require amputation
Systemic chemotherapy and radiation when
margins are positive
Prognosis
5 year survival rate – 40-50%
Lung is most common site of metastases
Metastatic Sarcomatoid Carcinoma of the Lung
Metastatic Sarcomatoid Carcinoma of the Lung

Keratin AE1,3 TTF1

Metastatic Spindle Cell Melanoma
Metastatic Sarcomatoid Carcinoma of the Kidney
 Case 10
Case Presentation

A 38 year old male complains of

buttock pain….
SMA +
S100+
EMA+