obsolete clear cell myomelanocytic tumor of the Falciform Ligament/Ligamentum Teres

Clear Cell Myomelanocytic Tumor of the Falciform Ligament/Ligamentum Teres

The Clear Cell Myomelanocytic Tumor of the Falciform Ligament/Ligamentum Teres is a rare and obsolete tumor entity. It was previously considered a distinct type of clear cell myomelanocytic tumor, but it has since been reclassified.

Characteristics:

• This tumor is characterized by the presence of clear spindle cells arranged in fascicles and nests.
• It typically affects young girls.
• The tumor usually presents with a predilection for children and young adults.

Definition:

The Clear Cell Myomelanocytic Tumor of the Falciform Ligament/Ligamentum Teres is defined as a very rare, usually benign neoplasm with perivascular epithelioid cell differentiation. It is characterized by the presence of clear spindle cells arranged in fascicles and nests.

References:

• [1] The term "Clear Cell Myomelanocytic Tumor of the Falciform Ligament/Ligamentum Teres" was proposed for this tumor entity.
• [2-3] This tumor is considered obsolete, with no further references or updates available.
• [4] A novel member of the perivascular epithelioid clear cell family of tumors with a predilection for children and young adults.

Citations:

[1], [15]

Signs and Symptoms of Obsolete Clear Cell Myomelanocytic Tumor of the Falciform Ligament/Ligamentum Teres

The signs and symptoms of obsolete clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres are not always present, but when they occur, they can be site-dependent. According to various medical sources [1][5], some common manifestations include:

• Swelling at the very end of a long bone
• Abdominal pain with radiation to the right upper quadrant (as mentioned in symptom 33)
• Iron deficiency anemia

It's worth noting that clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres is a rare hepatic tumor, and its signs and symptoms may not always be present or easily identifiable [14].

References:

[1] Context 4 [5] Context 5 [14] Context 15

To determine the diagnostic tests for Clear Cell Myomelanocytic Tumor (CCMMT) of the Falciform Ligament/Ligamentum Teres, several studies and reports have been consulted.

Diagnostic Tests

A diagnosis of CCMMT of ligamentum teres/falciform ligament may involve the following tests and procedures:

• Complete physical exam with evaluation of medical history: If the tumor is associated with tuberous sclerosis; then,

Based on the search results, it appears that there are limited information available on the drug treatment for Clear Cell Myomelanocytic Tumor (CCMMT) of the Falciform Ligament/Ligamentum Teres. However, I can provide some relevant information from the search results.

• According to search result [5], malignant PEComa (which includes CCMMT) can be treated with mTOR inhibitors such as temsirolimus.
• Search result [6] mentions that treatment of malignant uterine PEComas with mTOR inhibitors was effective in two out of three patients after surgical resection, with durable response.

It is worth noting that the information available on CCMMT is limited and mostly related to its classification and characteristics. There is no specific mention of drug treatment for this particular type of tumor.

However, based on the general information available on PEComa and mTOR inhibitors, it can be inferred that:

• Treatment options for CCMMT may include surgical intervention with complete excision (search result [13]).
• mTOR inhibitors such as temsirolimus may be considered as a treatment option for malignant CCMMT.

Please note that this information is based on limited data and should not be taken as definitive or up-to-date medical advice. Further research and consultation with medical professionals are necessary to determine the most effective treatment options for CCMMT.

References:

[5] by A Zaidi · 2024 — Clear cell myomelanocytic tumor (CCMMT) of the falciform ligament/ligamentum teres is a rare liver tumor and is a variant of perivascular epithelioid cell ... [6] by KD STARBUCK · 2016 · Cited by 58 — Treatment of malignant uterine PEComas with mTOR inhibitors was effective in two out of three patients after surgical resection, with durable response. [13] The treatment measures for Clear Cell Myomelanocytic Tumors of Falciform Ligament/Ligamentum Teres may include the following: Surgical intervention with complete excision can result in a complete cure.

The differential diagnosis for clear cell myomelanocytic tumor (CCMMT) of the falciform ligament/ligamentum teres involves several conditions that can present similarly. According to various studies, the following are some of the key differential diagnoses:

• Clear cell sarcoma of tendons and aponeuroses: This is a rare type of soft tissue tumor that can be difficult to distinguish from CCMMT.
• Leiomyosarcoma: A malignant tumor of smooth muscle origin, which can also present in the falciform ligament/ligamentum teres.
• Angiomyolipoma (AML): A benign tumor composed of blood vessels and fat cells, which is a member of the perivascular epithelioid cell tumor (PEComa) family, like CCMMT.

These conditions can be distinguished from CCMMT through histopathological examination and immunohistochemistry. For instance:

• Clear cell sarcoma of tendons and aponeuroses typically shows a more uniform clear cell appearance under the microscope.
• Leiomyosarcoma is characterized by its spindle-shaped cells and positive staining for smooth muscle markers like h-caldesmon.
• Angiomyolipoma (AML) can be distinguished from CCMMT by its presence of fat cells and characteristic "sugar" tumor-like appearance.

It's worth noting that the differential diagnosis of CCMMT also includes other rare tumors, such as malignant perivascular epithelioid cell tumor of the retroperitoneum. However, these conditions are relatively rare and require a thorough clinical evaluation to distinguish from CCMMT.

References:

• [5] describes a case of PEComa in the abdominal cavity at the falciform ligament.
• [6] mentions that hepatic PEComas are quite rare and diagnosis involves positivity of Melan-A and HMB45 on immunohistochemistry.
• [7] discusses the 2013 WHO classification of soft tissue tumours, which integrates conventional histology and molecular genetics for diagnosis.
• [9] describes a case of clear cell myomelanocytic tumor (CCMMT) of the falciform ligament/ligamentum teres and mentions its differential diagnosis with other epithelioid cell tumors.
• [10] proposes the term "clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres" for this rare condition and discusses its differential diagnosis with clear cell sarcoma of tendons and aponeuroses, leiomyosarcoma, and angiomyolipoma (AML).