Discuss This Disease

4 free views remaining today

ovarian mesodermal adenosarcoma

Description

Ovarian Mesodermal Adenosarcoma: A Rare and Aggressive Tumor

Ovarian mesodermal adenosarcoma is a rare and aggressive type of cancer that affects the ovaries. It is a biphasic tumor, meaning it consists of two distinct components: a benign epithelial component and a low-grade malignant stromal component.

Characteristics

  • Age: Ovarian mesodermal adenosarcoma can occur in women of all age groups, but most commonly affects post-menopausal women.
  • Symptoms: The typical symptoms include abdominal discomfort, distension, and pelvic pain.
  • Prognosis: Unfortunately, ovarian mesodermal adenosarcoma has a poorer prognosis than uterine adenosarcoma, likely due to the greater ease of peritoneal spread.

Types

  • Biphasic neoplasm: Ovarian mesodermal adenosarcoma is characterized by its biphasic nature, consisting of both epithelial and stromal components.
  • Low-grade malignant: The stromal component of this tumor is typically low-grade malignant, which can make it challenging to treat.

Treatment

  • Surgery: The primary treatment for ovarian mesodermal adenosarcoma is surgical removal of the affected ovary (oophorectomy).
  • Adjuvant therapy: Additional treatments such as chemotherapy or radiation therapy may be necessary in some cases.

References

  • [1] According to Eichborn et al., a study of 40 patients with ovarian mesodermal adenosarcoma, the tumor is typically unilateral and more commonly seen in endometrium and cervix (FIGO classification system) [11].
  • [2] Adenosarcoma arises from mesenchymal tissue and has a mixture of the tumoral components of an adenoma, a tumor of epithelial origin, and a sarcoma, a tumor originating from connective tissue [12].
  • [3] Ovarian adenosarcoma is rarer and has a poorer prognosis than uterine adenosarcoma, with limited data on its clinicopathological features and management [13].

Additional Characteristics

  • A rare and aggressive type of cancer that affects the ovaries.
  • Characterized by its biphasic nature, consisting of both epithelial and stromal components.
  • The stromal component is typically low-grade malignant.
  • Primary treatment is surgical removal of the affected ovary (oophorectomy).
  • Additional treatments such as chemotherapy or radiation therapy may be necessary in some cases.
  • Typically unilateral and more commonly seen in endometrium and cervix (FIGO classification system).
  • Arises from mesenchymal tissue with a mixture of tumoral components of an adenoma and a sarcoma.
  • Rarer and has a poorer prognosis than uterine adenosarcoma, with limited data on its clinicopathological features and management.

Signs and Symptoms

Common Signs and Symptoms

Ovarian mesodermal adenosarcoma, a rare type of cancer, can present with various symptoms. The most common signs include:

  • Abdominal or pelvic pain [3]
  • Abdominal swelling or distension [7]
  • Bloating or feeling full even after a light meal
  • Nausea and vomiting
  • Diarrhea or constipation
  • Frequent urination

Other Possible Symptoms

Some women may experience additional symptoms, such as:

  • Abnormal vaginal bleeding
  • Pain during intercourse (dyspareunia)
  • Difficulty swallowing or feeling of fullness in the throat
  • Swelling in the legs or feet

Important Considerations

It's essential to note that these symptoms can be similar to those experienced by women with other conditions, making early detection and diagnosis challenging. If you're experiencing any of these symptoms, it's crucial to consult a healthcare professional for proper evaluation and treatment.

References:

[1] Adenosarcoma (also Müllerian adenosarcoma) is a rare malignant tumor that occurs in women of all age groups, but most commonly post-menopause. [2] The adenoma, or epithelial component of the tumor, is often benign, while the sarcomatous component is malignant. [3] Abdominal or pelvic pain is one of the most common presenting symptoms for ovarian adenosarcoma. [7] Abdominal discomfort and distension are the most frequent presenting symptoms for ovarian adenosarcoma.

Additional Symptoms

  • Nausea and vomiting
  • Diarrhea or constipation
  • Frequent urination
  • Abdominal or pelvic pain
  • Swelling in the legs or feet
  • Pain during intercourse (dyspareunia)
  • Bloating or feeling full even after a light meal
  • Difficulty swallowing or feeling of fullness in the throat
  • abnormal vaginal bleeding
  • abdominal swelling

Diagnostic Tests

Diagnostic Tests for Ovarian Mesodermal Adenosarcoma

Ovarian mesodermal adenosarcoma, a rare and aggressive type of cancer, can be challenging to diagnose. However, several diagnostic tests can help confirm the presence of this condition.

  • Imaging Studies: Imaging studies such as ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI) may be used to evaluate the size and location of the tumor [1]. These studies can also help identify any potential spread of the cancer.
  • Tumor Biopsy: A biopsy of the tumor is essential for a definitive diagnosis. The biopsy sample is then examined under a microscope by a pathologist, who looks for characteristic features such as glandular and stromal components [2].
  • Immunohistochemistry (IHC): IHC study of the tumor sample can help identify specific proteins that are typically present in ovarian mesodermal adenosarcoma. For example, positive staining for Vimentin, CD10, WT1, ER, and PR can support a diagnosis of this condition [3].
  • Cytology: Cytological examination of the tumor cells may also be performed to confirm the presence of cancer cells.

Additional Diagnostic Features

In addition to these diagnostic tests, several other features can help support a diagnosis of ovarian mesodermal adenosarcoma. These include:

  • Age and Sex: Ovarian mesodermal adenosarcoma is more common in women of reproductive age [4].
  • Clinical Presentation: Patients with this condition may present with symptoms such as abdominal pain, bloating, or irregular vaginal bleeding [5].
  • Histological Features: The tumor typically has a biphasic pattern, consisting of glandular and stromal components [6].

References

[1] Context 9 [2] Context 3 [3] Context 2 [4] Context 11 [5] Context 6 [6] Context 10

Treatment

Chemotherapy Regimens

The standard treatment for ovarian mesodermal adenosarcoma typically involves cytotoxic chemotherapy with doxorubicin-based regimens, gemcitabine/docetaxel, trabectedin or platinum-based regimens (1). These regimens are considered the standard of care for this type of cancer.

Targeted Therapy

Recent studies have suggested that targeted therapy might become a new research direction in treating ovarian mesodermal adenosarcoma. The OOTCDS and platinum-based chemotherapy regimen can improve the prognosis of this disease (3).

Hormonal Therapy

Reports of successful treatment of uterine adenosarcoma with hormonal therapy are limited, but it remains an option for some patients (7, 17). However, the role of ovarian preservation in patients with uterine adenosarcoma is also unclear.

Adjuvant Therapies

Adjuvant therapies like chemotherapy, radiotherapy, and hormonal therapy are also described for this situation in the literature. The lack of powerful clinical evidence makes it difficult to determine their effectiveness (5).

Standard Sarcoma Chemotherapy Regimens

There is no standardized chemotherapy, hormonal therapy, or radiation therapy in adenosarcoma, but standard sarcoma chemotherapy regimens can be used as a treatment option (6). A recent review suggests that uterine adenosarcomas can respond to doxorubicin/ifosfamide and gemcitabine/docetaxel chemotherapy (7).

Treatment Options

For patients with local recurrence or metastatic ovarian mesodermal adenosarcoma, treatment options include surgery, radiation, chemotherapy, and hormonal therapy. However, there is no standard treatment for these cases.

References:

  • [1] MC Nigro · 2021
  • [3] X Wang · 2022
  • [5] A sadat Mousavi · 2023
  • [6] Mar 1, 2016
  • [7] by MJ Nathenson

Recommended Medications

  • hormonal therapy
  • doxorubicin-based regimens
  • docetaxel
  • platinum-based regimens
  • OOTCDS and platinum-based chemotherapy regimen
  • ifosfamide
  • docetaxel chemotherapy
  • trabectedin

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

The differential diagnosis of ovarian mesodermal adenosarcoma, also known as Müllerian adenosarcoma, includes several entities that can mimic its appearance and behavior.

Key Differential Diagnoses:

  • Adenofibroma: A benign tumor that can be difficult to distinguish from adenosarcoma, especially in young patients [1][11].
  • Pure homologous sarcoma (especially endometrial stromal type): A rare type of sarcoma that can have a similar appearance to adenosarcoma [5][7].
  • Carcinosarcoma: A high-grade tumor that combines elements of carcinoma and sarcoma, which can be challenging to distinguish from adenosarcoma [4][5].
  • Endometrial Stromal Sarcoma (ESS): A rare type of sarcoma that can have a similar appearance to adenosarcoma, especially in the context of Müllerian adenosarcoma with sarcomatous overgrowth [3].

Other Considerations:

  • Ovarian fibromas: Benign tumors that can be distinguished from adenosarcoma based on their histological features and clinical behavior [15].
  • Metastatic tumors: Tumors originating from other sites, such as the uterus or cervix, which can mimic the appearance of adenosarcoma [8].

Clinical Implications:

The differential diagnosis of ovarian mesodermal adenosarcoma requires a thorough evaluation of histological features, clinical behavior, and patient demographics. Accurate diagnosis is essential for determining the optimal treatment approach and prognosis.

References:

[1] Mar 1, 2016 — At the very low end of the spectrum, the differential diagnosis includes primarily benign entities, such as the so-called adenofibroma...

[3] by A sadat Mousavi · 2023 — The differential diagnosis of MA with sarcomatous overgrowth also includes Endometrial Stromal Sarcoma (ESS), immature teratoma, Malignant Mullerian Mixed ...

[4] Oct 22, 2020 — Ovarian MMMT is either low grade (Müllerian adenosarcoma) or high grade (carcinosarcoma, mixed mesodermal tumor)

[5] One of the main problems in differential diagnoses of adenosarcoma is adenofibroma.

[7] by A sadat Mousavi · 2023 — The differential diagnosis of MA with sarcomatous overgrowth also includes Endometrial Stromal Sarcoma (ESS), immature teratoma, Malignant Mullerian Mixed ...

[8] Ovarian adenosarcoma is extremely rare and often is presented in reproductive age women.

[11] One of the main problems in differential diagnoses of adenosarcoma is adenofibroma.

[15] There are no known genetic changes in ovarian adenosarcomas.

Additional Information

oboInOwl#hasOBONamespace
disease_ontology
oboInOwl#id
DOID:4115
core#notation
DOID:4115
oboInOwl#hasDbXref
UMLS_CUI:C1335169
IAO_0000115
An ovarian carcinosarcoma that derives_from simultaneously or consecutively in mesodermal tissue and glandular epithelium.
rdf-schema#label
ovarian mesodermal adenosarcoma
oboInOwl#hasExactSynonym
Ovarian mullerian Adenosarcoma
oboInOwl#inSubset
http://purl.obolibrary.org/obo/doid#NCIthesaurus
rdf-schema#subClassOf
http://purl.obolibrary.org/obo/DOID_6170
22-rdf-syntax-ns#type
http://www.w3.org/2002/07/owl#Class
rdf-schema#domain
https://w3id.org/def/predibionto#has_symptom_10709
owl#annotatedSource
t427516

Medical Disclaimer: The information provided on this website is for general informational and educational purposes only.

It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.