Abdominal Desmoid Fibromatosis
Differential Diagnosis
- Scar
- Nodular fasciitis
- Proliferative myositis
- Desmoplastic fibroblastoma
- Intramuscular myxoma
- Plexiform fibrous histiocytoma
- Inflammatory myofibroblastic tumor
- Fibrosarcoma
- Low grade myxofibrosarcoma
- Desmoplastic melanoma
- Calcifying fibrous pseudotumor
| Fibromatosis, Abdominal Desmoid, Extra-abdominal Desmoid, Mesenteric, Retroperitoneal and Pelvic | Scar |
|---|---|
| Uniform | Multiple stages of organization |
| Hemosiderin rare | Hemosiderin common |
| Rare under 3 cm | Rare over 3 cm |
| Inflammation not prominent | Inflammation may be prominent |
| Usually infiltrates muscle | Infiltration of muscle uncommon |
| Fibromatosis, Abdominal Desmoid and Extra-abdominal Desmoid | Nodular Fasciitis |
|---|---|
| Usually dense collagenous stroma | Collagenous stroma focal or rare |
| Linear fascicles of cells | Undulating bundles of cells |
| Infrequent mitotic figures | Frequent mitotic figures |
| Usually >3 cm | Usually <4 cm |
| Beta-catenin 80-90% | Beta-catenin negative |
| Fibromatosis, Abdominal Desmoid and Extra-abdominal Desmoid | Proliferative Myositis |
|---|---|
| Muscle fibers damaged or obliterated | Muscle fibers not damaged |
| Muscle fibers at edge of lesion | Muscle fibers throughout |
| No ganglion-like cells | Ganglion-like cells present |
| Predominantly collagenous | Predominantly myxoid |
| Usually >3 cm | Usually <4 cm |
| Fibromatosis, Abdominal Desmoid and Extra-abdominal Desmoid | Desmoplastic Fibroblastoma |
|---|---|
| Mild to moderate cellularity | Paucicellular |
| Moderate vascularity | Hypovascular |
| Extensive infiltration of muscle | Only focal muscle infiltration |
| Spindle cells usually in fascicular pattern | Spindle and stellate cells randomly aranged |
| Fibromatosis, Abdominal Desmoid and Extra-abdominal Desmoid | Intramuscular Myxoma |
|---|---|
| Mild to moderate cellularity | Markedly hypocellular |
| Areas with collagen | No collagen |
| Prominent vessels | Sparse vessels |
| Fibromatosis, Abdominal Desmoid and Extra-abdominal Desmoid | Plexiform Fibrous Histiocytoma |
|---|---|
| Deep to subcutis, in muscle | Superficial |
| Generally solid mass | Plexiform architecture |
| Rare under 3 cm | Rare over 3 cm |
| Giant cells and histiocytes rare | Nodules of giant cells and histiocytes |
| Fibromatosis, Abdominal Desmoid, Extra-abdominal Desmoid, Mesenteric, Retroperitoneal and Pelvic | Inflammatory Myofibroblastic Tumor |
|---|---|
| Rare <12 years of age | Age usually <14, rare >30 |
| Inflammation not prominent | Prominent inflammation, particularly plasma cells |
| Alk1 negative | Alk1 frequently positive |
| Beta catenin 80-90% | Beta catenin negative |
| Fibromatosis, Abdominal Desmoid and Extra-abdominal Desmoid | Adult Fibrosarcoma |
|---|---|
| Mild to moderate cellularity | Usually more cellular |
| Fine chromatin | Usually clumped chromatin |
| Rarely >5 mitotic figures / hpf | Usually >5 mitotic figures / hpf |
| Rarely necrotic | Frequent necrosis |
| Fibromatosis, Abdominal Desmoid, Extra-abdominal Desmoid, Mesenteric, Retroperitoneal and Pelvic | Low Grade Myxofibrosarcoma |
|---|---|
| No pleomorphism | Cytologic pleomorphism present |
| Usually dense stroma but focally may be myxoid | No dense stroma |
| Vessels lack arching pattern | Arching vessels |
| Fibromatosis, Abdominal Desmoid and Extra-abdominal Desmoid | Desmoplastic Melanoma |
|---|---|
| Based below subcutis, involves muscle | Usually based in dermis |
| S100 negative | S100 positive |
| Fibromatosis, Abdominal Desmoid and Extra-abdominal Desmoid | Calcifying Fibrous Pseudotumor |
|---|---|
| Nearly always has cellular areas | Uniformly paucicellular |
| Infiltrative pattern prominent | Microscopically infiltrative at most |
| No calcification | Calcification present |
| Inflammation uncommon | Chronic inflammation present |

