Ossifying Fibromyxoid Tumor of Soft Parts

DEFINITION AND PATHOGENESIS

• A tumor of unknown origin

IMPORTANCE

• No bony counterpart has been reported
• Intermediate malignant potential counterpart must be ruled out

CLINICAL FEATURES

• Usually present as a slow growing, small painless, well circumscribed subcutaneous or muscular mass
  • Has been reported presenting as a scalp cyst
• M:F = 2:1
• Median age 50 yrs (21-81)
  • Reported in the subcutaneous tissue of the head of a 2-yr-old
  • Retroperitoneal location reported ina 6-yr-old girl with paraspinal extension and disease free 9 yrs after resection
• Median duration of sxs = 4 yrs
• ?? alk?

RADIOLOGIC FEATURES

• Radiographs may be normal
  • Peripheral ossification can occur
• ? uptake on BS
• MRI with a well defined mass, with heterogeneous signal
• Unusual location
  • Ethmoid sinus
  • Parotid/zygomatic arch region
  • Mediastinum

GROSS PATHOLOGY

• Tumor size 0.7-17 cm (median 3- cm)
• Fibrous capsule with incomplete peripheral shell of mature bone reported

HISTOLOGIC AND MOLECULAR FEATURES

• Round to oval cells in cords or nests within a myxoid matrix ± transition to hyaline fibrosis and focal osteoid
  • Few or no mitotic activity
    • Higher mitotic activity reported (suggests the malignant counterpart
      • Mitotic activity reported from 0-50hpf
  • ± necrosis (17%) (suggests the malignant counterpart
  • ± vascular invasion (11%) (suggests the malignant counterpart
  • ± stromal cyst formation
• Incomplete capsular shell of mature bone
• Moderate amounts of myxocollagenous matrix
• ± focal peripheral metaplastic bone formation
  • Central zone bone formation has been reported
  • 40% with bone present
• Background cells can include histiocytes, lymphocytes, and mast cells
• Immunohistochemisty
  • + S-100 in 6094% reported
  • Pancytokeratin (10%)
  • SMA (%)
  • Desmin (13%)
  • Collagen II (%)
  • Glial fibrillary acidic protein positivity reported
  • Vimentin positivity reported
  • Neurofilament and neuron specific enolase positivity reported suggesting neural origin
  • + synaptophysin reported
• EM
  • Abundant intermediate filaments, primitive cell junctions, focally present external lamina reported (suggesting neural origin)
  • Cytoplasmic dense-core granules measuring 100-200nm
• Ctyogenetics
  • 42 approximately 46,XY,-Y,add(1)(q42),add(6)(p21),t(10;18)(q26;q11),der(11)t(11;15)(q23;q15),add(12)(q13),ins(14;?)(q13;?),-15,+mar reported
  • 45,XY, der(6;14)(p10;q10),add(12)(q24.3) reported

DIFFERENTIAL CLINICOPATHOLOGIC DIAGNOSIS

• Calcifying hematoma
• Aponeurotic fibroma
• Nodular fasciitis
• Neurilemoma
  • Epithelioid" type
• Myoepithelioma
• Mesenchymal hamartoma
• Ossifying epithelioid hemangioendothelioma
• Chondromyxoid fibroma
• Osteosarcoma of soft tissue
• Malignant schwannoma
• Low-grade fibromyxoid sarcomas
• Malignant ossifying fibromyxoid tumor (the malignant counterpart

DISEASE COURSE AND TREATMENT

• 22-30% LR (may result in amputation)(esp in large tumors, ? cellularity, ? mitoses)
  • LR usu ? 10 yrs
    • Subcutaneous LR reported
    • Chest wall LR reported after only 1 yr
  • > 2 mitotic figures/50 HPFs risk for LR
• Wide resection with clear margins indicated because of the indistinct differentiation between the benign and malignant counterparts
• One report associated with pulmonary metastases extending into the L atrium has been reported representing the malignant counterpart

FIGURES

There are no images attached to this page.

REFERENCES

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