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lymphoplasmacyte-rich meningioma
Description
Lymphoplasmacyte-Rich Meningioma (LPRM): A Rare Histologic Subtype
Lymphoplasmacyte-rich meningioma (LPRM) is a rare histologic subtype of benign (grade 1) meningiomas characterized by prominent infiltration of plasma cells and lymphocytes, with a variable proportion of meningothelial elements [12][13]. This variant of meningioma usually causes significant peritumoral brain edema and can mimic higher-grade lesions [12].
Key Features:
- Rare histologic subtype: LPRM is one of the rarest variants of grade I meningiomas, with only a few cases globally reported in the literature [9].
- Prominent infiltration: The tumor is characterized by conspicuous infiltration of plasma cells and lymphocytes, which over-shadow the underlying meningothelial component [13][14].
- Variable proportion: A variable proportion of meningothelial tumorous elements are present in LPRM [14].
- Grade I tumor: LPRM is classified as a grade I tumor in the World Health Organization (WHO) classification of tumors of central nervous system [14].
Clinical Associations:
- Peripheral blood abnormalities: LPRM can be clinically associated with prominent peripheral blood abnormalities, anemia, and/or various gammopathy, which usually disappear after surgical removal of the tumor [15].
- Significant peritumoral brain edema: The tumor is often associated with significant peritumoral brain edema, which can mimic higher-grade lesions [12].
References:
[9] X Yang et al. (2018) - Lymphoplasmacyte-rich meningioma is a rare variant of meningioma and is categorized as a WHO grade I tumor.
[12] Y Zhang et al. (2022) - Lymphoplasmacyte-rich meningioma is a rare form of meningioma that can cause significant peritumoral brain edema.
[13] Lymphoplasmacyte-rich meningioma (LPRM) is a rare pathological entity belong to the World Health Organization Grade I meningiomas.
[14] Lymphoplasmacyte-rich (LPR) meningioma is a rare, benign variant of meningioma, which is characterized by conspicuous infiltration of plasma cells and lymphocytes and a variable proportion of meningothelial tumorous elements.
[15] Lymphoplasmacyte-rich meningioma (LPRM) is one of the rarest variants of grade I meningiomas.
Additional Characteristics
- Rare histologic subtype
- Prominent infiltration of plasma cells and lymphocytes
- Variable proportion of meningothelial tumorous elements
- Grade I tumor in WHO classification
- Peripheral blood abnormalities, anemia, gammopathy associated with the tumor
- Significant peritumoral brain edema mimicking higher-grade lesions
Signs and Symptoms
Diagnostic Tests
Diagnostic Tests for Lymphoplasmacyte-Rich Meningioma
Lymphoplasmacyte-rich meningioma (LPRM) is a rare variant of meningioma, and its diagnosis can be challenging due to its unique characteristics. Here are some diagnostic tests that may be used to diagnose LPRM:
- Imaging studies: MRI and CT scans can help identify the tumor's location, size, and relationship with surrounding structures [8]. However, these imaging features may not be distinctive enough to differentiate LPRM from other types of meningiomas or even other brain tumors.
- Histopathological examination: A definitive diagnosis of LPRM requires histopathological examination of a tissue sample obtained through biopsy or surgical resection. The presence of lymphoplasmacytic infiltrates, plasma cells, and variable proportions of meningothelial elements are characteristic features of this tumor [14].
- Immunohistochemistry testing: Immunohistochemistry (IHC) testing can help confirm the diagnosis by identifying specific markers such as epithelial membrane antigen (EMA), CD20, and SSTR2a [7][10]. These markers can be used to differentiate LPRM from other types of meningiomas or even other brain tumors.
- Blood tests: Blood tests may reveal abnormalities such as anemia and polyclonal gammopathy, which can normalize following resection [5].
- Dural biopsy: In some cases, a dural biopsy may be necessary to confirm the diagnosis of LPRM.
References:
[7] by J Li · 2021 · Cited by 4 — Immunohistochemistry testing showed epithelial membrane antigen positive (arrowheads) (magnification, ×400) (B), CD20 positive (arrowheads) ( ...
[8] by X Yang · 2018 · Cited by 8 — Lymphoplasmacyte-rich meningioma is a rare histologic subtype of benign (grade 1) meningiomas characterized by prominent infiltration of plasma cells and lymphocytes, with a variable proportion of meningothelial elements.
[10] 10. The diagnosis of LPRM was confirmed by dural biopsy.
[14] Lymphoplasmacyte-rich meningioma is a rare histologic subtype of benign (grade 1) meningiomas characterized by prominent infiltration of plasma cells and lymphocytes, with a variable proportion of meningothelial elements.
Treatment
Current Understanding of Drug Treatment for Lymphoplasmacyte-Rich Meningioma
Lymphoplasmacyte-rich meningioma (LPRM) is a rare subtype of meningioma, and its treatment options are limited due to its rarity. However, recent studies have explored potential drug treatments for this condition.
Surgical Excision as the Primary Treatment While surgical excision remains the primary treatment for LPRM, there is growing interest in exploring alternative or adjunctive treatments, including drugs, to improve patient outcomes.
Immunotherapy as a Potential Option According to [4], immunotherapy may be a new treatment option for LPRM. This approach aims to harness the body's immune system to fight cancer cells. However, more research is needed to determine its efficacy and safety in treating this rare condition.
Targeted Agents and Chemoradiotherapy Several promising targeted agents have been developed and are being investigated as potential therapeutic strategies for LPRM [13]. These agents aim to target specific molecular pathways involved in the growth and progression of meningiomas. Additionally, chemoradiotherapy may be considered in some cases, although its effectiveness is still being evaluated.
Pembrolizumab and Other Drugs Research has also explored the use of pembrolizumab, a checkpoint inhibitor, for the treatment of recurrent meningioma [5]. While this study did not specifically focus on LPRM, it highlights the potential for immunotherapy in treating meningiomas. However, more research is needed to determine its efficacy and safety in treating LPRM.
Current Challenges Despite these emerging treatment options, there are currently no effective treatments available for LPRM with extensive lesions and diffuse growth [9]. Further research is necessary to develop targeted therapies that can improve patient outcomes for this rare condition.
In summary, while surgical excision remains the primary treatment for LPRM, researchers are exploring alternative or adjunctive treatments, including immunotherapy, targeted agents, and chemoradiotherapy. However, more research is needed to determine their efficacy and safety in treating this rare subtype of meningioma.
Recommended Medications
- Immunotherapy
- Surgical Excision
- Pembrolizumab
- Chemoradiotherapy
- Targeted Agents
💊 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
Additional Information
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