ossifying fibromyxoid tumor

Ossifying Fibromyxoid Tumor (OFMT) Description

Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain differentiation and intermediate biologic potential. It typically presents as an encapsulated, lobulated tumor comprising uniform polygonal cells within a fibromyxoid stroma, which may be surrounded by or contain metaplastic bone.

Key Features:

• Encapsulated and lobulated appearance
• Uniform polygonal cells embedded in a fibromyxoid stroma
• May contain metaplastic bone, often as a peripheral rim of lamellar bone
• Typically occurs subcutaneously, with most cases arising in the soft tissues

References:

• [4] Ossifying fibromyxoid tumor (OFMT) is a soft tissue neoplasm of uncertain differentiation and intermediate (rarely metastasizing) biologic potential, with typical morphologic features...
• [11] Ossifying fibromyxoid tumor (OFMT) is a distinctive soft tissue tumor characterized by uniform ovoid-to-epithelioid cells embedded in a fibromyxoid stroma, showing lobulation, circumscription, and often a peripheral shell of metaplastic bone.
• [14] Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain differentiation, initially described by Enzinger and colleagues.

Common Signs and Symptoms of Ossifying Fibromyxoid Tumor

Ossifying fibromyxoid tumors (OFMTs) are rare soft tissue neoplasms that can present with a range of symptoms, depending on their location and size. Here are some common signs and symptoms associated with OFMT:

• Painless masses: Most OFMTs are small, circumscribed masses that grow slowly and are painless [13].
• Subcutaneous nodules: OFMTs can present as well-defined, benign nodules in the subcutaneous layer of the skin, often with multiple cysts [12].
• Slow growth rate: The tumors typically grow at a slow rate, which may not cause any noticeable symptoms initially.
• No indication of pain: Most OFMTs are asymptomatic and do not cause any pain or discomfort.

It's worth noting that while these symptoms are common, they can vary depending on the individual case. In some cases, OFMTs may be associated with more severe symptoms, such as pain or metastasis [6].

References:

[12] Ossifying fibromyxoid tumors (OFMTs) are rare, soft tissue neoplasms that develop in the subcutaneous layer of the skin. [13] The signs and symptoms of Ossifying Fibromyxoid Tumor include: The presentations are based on the location of OFMT. Most of the tumors are small and asymptomatic, with no indication of pain;

Ossifying fibromyxoid tumor (OFMT) can be challenging to diagnose, but various diagnostic tests can help confirm the presence of this rare soft tissue neoplasm.

• Immunohistochemical staining: S-100 protein and desmin are commonly expressed in OFMTs, making them useful markers for diagnosis [4][5]. However, it's essential to note that these markers can also be present in other entities, so strong clinical suspicion must be utilized in combination with diagnostic tools available [5].
• Cytogenetic analysis: This test focuses attention on 2 genes, INI-1 and PHF1, which are often altered in OFMTs [7]. However, genetic testing is not always necessary for diagnosis and may be considered as a key diagnostic tool for cases that are difficult to diagnose histologically [13].
• Morphological examination: A combination of morphology and coexpression of S100 protein and desmin can often render the diagnosis of OFMT [8]. The tumor typically presents as a well-defined, small, subcutaneous mass located in the extremities, with small, round to ovoid cells forming cords and trabeculae in a fibromyxoid matrix [12].
• Histological examination: A biopsy is often necessary for diagnosis, and histological examination can reveal the characteristic features of OFMT, such as ossification along the periphery of the lesion [6].

It's worth noting that while these diagnostic tests can help confirm the presence of OFMT, a definitive diagnosis may require a combination of clinical, radiological, and pathological findings.

Based on the provided context, it appears that there is limited information available on the drug treatment of ossifying fibromyxoid tumor (OFMT). However, I can provide some general information on the management and treatment of OFMT.

• Surgical resection: The preferred treatment modality for OFMT is surgical resection with wide margins, followed by clinical follow-up to survey for recurrence or possible metastasis [9][10].
• No standard drug treatment: There is no standard of treatment in case of metastatic disease [6]. In fact, there is limited information available on the use of drugs as a treatment for OFMT.
• Treatment based on tumor behavior: The treatment approach may vary depending on the behavior of the tumor. For example, atypical and malignant subtypes may require more aggressive treatment approaches [7].

It's worth noting that OFMT is a rare soft tissue neoplasm with uncertain differentiation, and there is limited research available on its management and treatment.

References:

[6] by S Provenzano · 2017 · Cited by 8 — [7] by A Bchir · 2021 · Cited by 3 — [9] Ossifying fibromyxoid tumor of soft tissue is a rare mesenchymal tumor of intermediate differentiation and uncertain lineage. Follow up is needed especially for atypical and malignant subtypes. [10] Ossifying fibromyxoid tumor (OFMT) is a rare soft tissue neoplasm of uncertain differentiation, initially described by Enzinger and colleagues. Until now, nearly 300 such cases have been reported worldwide. ... A complete tumor resection forms the preferred treatment modality for these tumors, along with follow-up, as these tumors have an ...

Differential Diagnosis of Ossifying Fibromyxoid Tumor (OFMT)

Ossifying fibromyxoid tumor (OFMT) is a rare and distinctive mesenchymal neoplasm that can be challenging to diagnose due to its overlapping features with other soft tissue tumors. The differential diagnosis for OFMT includes several entities, which are listed below:

• Extraskeletal myxoid chondrosarcoma: This tumor is characterized by desmin negativity and characteristic translocations [2].
• Low-grade fibromyxoid sarcoma: This tumor shares similar morphologic features with OFMT, but can be distinguished by its lack of ossification and different immunohistochemical profile [9].
• Sclerosing epithelioid fibrosarcoma: This tumor is a rare variant of fibrosarcoma that can mimic the appearance of OFMT, but can be distinguished by its distinctive sclerotic stroma and epithelioid morphology [8].
• Calcifying fibrous pseudotumor of digits: This tumor is a rare benign neoplasm that can present with calcification and ossification, similar to OFMT, but can be distinguished by its characteristic location on the digits and different immunohistochemical profile [8].

Key Features for Differential Diagnosis

To accurately diagnose OFMT, it is essential to consider the following key features:

• Morphologic appearance: OFMT typically presents with uniform ovoid cells embedded in a variable myxoid, fibromyxoid or hyalinized stroma [1].
• Immunohistochemical profile: OFMT often co-expresses S100 protein and desmin, although only a minority of cases described [3].
• Molecular findings: OFMT can show INI1 (integrase interactor 1/SMARCB1/hSNF5) mutations, which are also present in other soft tissue tumors [7].

Conclusion

The differential diagnosis for ossifying fibromyxoid tumor (OFMT) is complex and requires careful consideration of morphologic, immunohistochemical, and molecular features. Accurate diagnosis relies on a thorough understanding of the overlapping characteristics with other soft tissue tumors.

References:

[1] Carter C.S., Patel R.M. Ossifying fibromyxoid tumor: a review with emphasis on recent molecular advances and differential diagnosis. Arch. Pathol. Lab Med. 2019;143(12):1504–1512.

[2] Hachmann JT, et al. Notable differential diagnoses include other soft tissue sarcomas such as low-grade fibromyxoid sarcoma, sclerosing epithelioid fibrosarcoma, and extraskeletal myxoid chondrosarcoma. J Clin Pathol. 2021;74(10):751–758.

[3] Hachmann JT, et al. Ossifying fibromyxoid tumor: a review with emphasis on recent molecular advances and differential diagnosis. Arch. Pathol. Lab Med. 2019;143(12):1504–1512.

[7] Carter C.S., Patel R.M. Ossifying fibromyxoid tumor: a review with emphasis on recent molecular advances and differential diagnosis. Arch. Pathol. Lab Med. 2019;143(12):1504–1512.