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Soft Tissue Pathology 

Pathology of Low-Grade Fibromyxoid Sarcoma ;

Pathology of Hyalinizing Spindle Cell Tumour with Giant Rosettes

Dr Sampurna Roy MD       

Dermatopathology Quiz Case 173 

Diagnosis: Hyalinizing Spindle Cell Tumour with Giant Rosettes

Dermatopathology Quiz Case 167

Diagnosis: Low-Grade Fibromyxoid Sarcoma

 

                                                                                                                      

 

 

Pathology of Low-Grade Fibromyxoid Sarcoma:

Low-grade fibromyxoid sarcoma was first described by Evans in 1987.Low-grade fibromyxoid sarcoma. A report of two metastasizing neoplasms having a deceptively benign appearance.

This is an aggressive tumour and local recurrences have been reported in approximately 10% of the cases, and distant spreading occurs in 5-10% of the cases.

Distant metastasis occurs mostly to the lungs.

Age: The tumour occurs in the third and fifth decade of life. (Average: 35 years)

Site: This is a neoplasm of the deep and subcutaneous soft tissue and are commonly located in the lower extremities.

Other sites include neck, axilla, chest wall, shoulder, buttock and the inguinal region.

The tumour may be rarely located in the mediastinum, retroperitoneum, mesentery and the pelvis.

Gross features: The tumours are round to oval shaped and well circumscribed with thin pseudocapsule.

The cut surface is homogenous with a firm and fibrous consistency.

Microscopic features:

Lee EJ, Hwang HJ, Byeon HK, Park HS, Choi H-S. A low grade fibromyxoid sarcoma originating from the masseter muscle: a case report. Journal of Medical Case Reports. 2015;9:176. doi:10.1186/s13256-015-0658-9.

The tumour consists of bland uniform fibroblasts arranged  in a swirling, whorled growth pattern.

The cells are small, spindle to stellate shaped with poorly defined, palely eosinophilic cytoplasm and hyperchromatic ovoid nuclei

These cells are embedded in a dense collagenous or myxoid stroma.

Cellularity is low to moderate and mitotic figures are rare. The recurrent tumours are usually more cellular.

The hyalinizing spindle cell tumour with giant rosettes is thought to be a closely related tumour differing only by the presence of collagen rosettes.

Immunohistochemistry:  The tumour cells are strongly positive for vimentin. 

There may be focal immunopositivity for actin, desmin & cytokeratin. CD34 and S100 protein are usually negative.

Low-grade fibromyxoid sarcoma is characterized by a recurrent balanced translocation t (7 ; 16) (q34; p11) resulting in an FUS-CREB3L2 fusion gene.

This same translocation is identified in cases of hyalinizing spindle cell tumor with giant rosettes, suggesting a pathogenetic link between these two entities.

Differential diagnosis :

Visit: Myxoid Tumours of Soft Tissue ;  Fibromatosis ;  Myxoid neurofibroma ;  Perineuroma ;  Nodular fascitis ;

Low- grade myxofibrosarcoma is characterized by the presence of prominent elongated, curvilinear capillaries and pseudolipoblasts, accompanied by an abundant myxoid matrix.

It has no extensive solid areas.

Low-grade fibromyxoid sarcoma is composed of bland spindle cells arranged in a whorled pattern with alternating myxoid and fibrous stroma.

Curvilinear capillaries are not prominent and cytological atypia is absent.

Malignant peripheral nerve sheath tumour ; Spindle cell liposarcoma;

 

Further reading

Low-Grade Fibromyxoid Sarcoma: Incidence, Treatment Strategy of Metastases, and Clinical Significance of the FUS Gene

The clinical significance of the FUS-CREB3L2 translocation in low-grade fibromyxoid sarcoma

Low-grade fibromyxoid sarcoma of the hand: a case report

Haemorrhagic low-grade fibromyxoid sarcoma: MR findings in two young women

Low Grade Fibromyxoid Sarcoma in Thigh

A case of low-grade fibromyxoid sarcoma with unusual central necrosis in a 77-year-old man confirmed by FUS-CREB3L2 gene fusion

Multiple Low-Grade Fibromyxoid Sarcoma on the Upper Arms with Atypical Histological Presentation

Low grade fibromyxoid sarcoma: a case report and review of the literature

Low-Grade Fibromyxoid Sarcoma of the Malar Area

Superficial low-grade fibromyxoid sarcoma (Evans tumor): a clinicopathologic analysis of 19 cases with a unique observation in the pediatric population.

Spectrum of low-grade fibrosarcomas: a comparative ultrastructural analysis of low-grade myxofibrosarcoma and fibromyxoid sarcoma.

Low-grade fibromyxoid sarcoma versus low-grade myxofibrosarcoma in the extremities and trunk. A comparison of clinicopathological and immunohistochemical features.

 

Pathology of Hyalinizing Spindle Cell Tumour with Giant Rosettes:

This tumour was first reported by Lane et al in 1997. Hyalinizing spindle cell tumor with giant rosettes: a distinctive tumor closely resembling low-grade fibromyxoid sarcoma.Am J Surg Pathol. 1997 Dec;21(12):1481-8.

It is now regarded as a variant of low-grade fibromyxoid sarcoma.

It is a rare low-grade sarcoma with indolent behavior, and wide resection of the tumour with prolonged follow-up are needed because patients may develop late metastases.

Age: This painless, slow growing tumour can develop at any age.

It commonly occurs in young and middle aged adults (average age 38 years).

Site:   The lesion is commonly located in the deep soft tissue of the extremities, particularly the thigh. Other sites include the chest wall, axilla, buttock and the neck.     

Gross features:  These are deep seated, oval, multilobulated , well circumscribed, pale, firm tumours.

The lesion usually ranges from 5-10 cm in maximal dimension.

Focal pseudocystic degeneration  may be present.

Microscopic features:

The tumour consists of uniform oval to spindle cells with fibroblastic or neural-like appearance.

These are arranged in fascicles in a collagenous or myxoid stroma.

The most striking feature is the presence of hyalinized nodules surrounded by round or ovoid tumour cells giving the appearance of rosette or palisaded granuloma.

The eosinophilic area at the centre of the rosettes is composed of collagen fibrils.

Pleomorphism is minimal. Mitoses are scarce.

In small number of cases there may be hypercellular areas with cells showing nuclear enlargement and hyperchromatism. 

The miototic activity may range from 1 to 20 per 10 high-power fields.

Immunohistochemistry: Spindle cells are immunoreactive for vimentin, HAM56factor XIIIa , collagen IV, and CD68.

The rounded cells comprising the collagen rosettes are positive for vimentin, S100 protein, Leu7(CD57) , synaptophysin, protein gene product (PGP) 9.5 , neuron specific enolase and sometimes CD34.

The cells are negative for desmin, alpha-smooth muscle actin , keratin, and epithelial membrane antigen.

Differential diagnosis:   Fibromatosis Fibrosarcoma ;  Neuroblastoma like neurilemmoma ;   Fibrosarcomatous DFSP with giant rosettes : 

 

 Further reading

Hyalinizing spindle cell tumor with giant rosettes with pulmonary metastasis after a long hiatus: a case report.

Hyalinizing spindle cell tumor with giant rosettes and low-grade fibromyxoid sarcoma: an immunohistochemical and ultrastructural comparative investigation.  

Hyalinizing spindle cell tumor with giant rosettes of the omentum. J Obstet Gynaecol Res. 2003 Dec;29(6):388-91

Hyalinising spindle cell tumour with giant rosettes: report of a case with unusual features including original histological and ultrastructural observations.

Hyalinizing spindle cell tumor with giant rosettes--a soft tissue tumor with mesenchymal and neuroendocrine features. An immunohistochemical, ultrastructural, and cytogenetic analysis.

Hyalinizing spindle cell tumor with giant rosettes: a report of three cases with ultrastructural analysis

Hyalinizing spindle cell tumors with giant rosette-like structures.

Hyalinizing spindle cell tumor with giant rosettes. Case report with immuno-histochemical characterization.

Hyalinizing spindle cell tumor with giant rosettes: report of a case showing remarkable myofibroblastic differentiation.

Hyalinizing spindle cell tumor with giant rosettes of the broad ligament.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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