liver leiomyosarcoma

Liver leiomyosarcoma is a rare type of cancer that originates from smooth muscle cells in the liver. It is considered a primary malignant tumor, meaning it begins in the liver rather than spreading to it from another location.

According to various sources [4][6], liver leiomyosarcomas are extremely rare and have been reported in only a few cases. They can grow large enough to cause symptoms such as abdominal pain or discomfort, but their presentation is often delayed, leading to relatively poor prognosis [8].

The pathological features of liver leiomyosarcoma include spindle-shaped cells, abundant cytoplasm, nuclear atypia, and the presence of mitotic figures [3]. This type of cancer can metastasize (spread) to other parts of the body, such as the lungs or liver, which is a frequent complication [13].

It's worth noting that liver leiomyosarcoma can be misdiagnosed as a liver abscess in rare instances, especially when it presents with fever [1][5]. Early detection and treatment are crucial for improving outcomes.

References: [1] by W Liu · 2018 · Cited by 13 — Hepatic leiomyosarcoma is a rare malignant tumor... [3] The pathological features of leiomyosarcoma include spindle-shaped cells, abundant cytoplasm, nuclear atypia, and the presence of mitotic figures. [4] May 16, 2024 — Hepatic leiomyosarcomas are rare primary malignant tumors derived from smooth muscle cells in the liver. [5] by W Liu · 2018 · Cited by 13 — Hepatic leiomyosarcoma is a rare malignant tumor... [6] by C Faraj · 2024 — Primary hepatic leiomyosarcoma (PHL) is an uncommon mesenchymal hepatic tumor which originates from smooth muscles. [8] Sep 27, 2022 — PHL is a rare malignant disease with often delayed presentation...

Liver leiomyosarcoma, a rare type of cancer, can be challenging to diagnose. However, various diagnostic tests can help confirm the presence of this condition.

Imaging Tests

• Computed Tomography (CT) scans: CT scans can show the size and location of the tumor in the liver, as well as any potential metastases [4]. A "pseudocystic pattern" with heterogeneous enhancement is commonly seen on CT scans [4].
• Magnetic Resonance Imaging (MRI): MRI scans can provide detailed images of the liver and surrounding tissues, helping to identify the tumor and assess its size and location [8].

Laboratory Tests

• Blood tests: Blood tests may be conducted to check for abnormal liver function or other related issues. However, these tests are not diagnostic on their own [2].
• Alpha-fetoprotein (AFP) levels: Elevated AFP levels can indicate the presence of a tumor in the liver, but this is not specific to leiomyosarcoma [2].

Biopsy

• Image-guided needle biopsy: A biopsy is usually the next step in diagnosing liver leiomyosarcoma. This involves taking a sample of the tumor tissue using an image-guided needle biopsy [3]. The sample is then examined under a microscope by a pathologist to confirm the presence of cancer cells.

Other Tests

• Ultrasound examination: An ultrasound may be used to initially identify the liver tumor, which can then be further evaluated with CT or MRI scans [6].
• Positron emission tomography (PET) scan: A PET scan may be conducted to assess the spread of the disease and evaluate potential metastases [10].

It's essential to note that a definitive diagnosis of liver leiomyosarcoma requires a biopsy, which involves taking a sample of the tumor tissue for examination by a pathologist.

Treatment Options for Liver Leiomyosarcoma

Liver leiomyosarcoma, a type of cancer that originates from smooth muscle cells in the liver, is a rare and aggressive disease. The treatment options for this condition are limited, but various therapies have been explored to improve patient outcomes.

• Chemotherapy: Chemotherapy drugs such as doxorubicin, ifosfamide, gemcitabine, and docetaxel have been used to treat liver leiomyosarcoma [1][2]. These medications can help shrink the tumor or slow its growth.
• Targeted Therapy: Tazemetostat, a targeted therapy drug, has shown promise in treating liver leiomyosarcoma by inhibiting the EZH2 protein, which is involved in cancer cell growth [4].
• Liver-Directed Therapies: Transarterial chemoembolization (DEB-TACE) and yttrium-90 (Y90) radioembolization are minimally invasive procedures that deliver chemotherapy or radiation directly to the tumor site [3]. These treatments can be effective in reducing tumor size and improving overall survival.
• Second-Line Treatment: For patients who have not responded to first-line therapy, second-line treatment options such as trabectedin, gemcitabine, or dacarbazine may be considered. These medications have shown some efficacy in achieving an objective response [14].

Current Challenges

The treatment of advanced leiomyosarcoma is challenging due to the limited effectiveness of current therapies and the high recurrence rate after surgical resection [15]. Further research is needed to develop more effective treatment strategies for this disease.

References:

[1] - Medical records of 24 patients with liver dominant metastatic leiomyosarcoma who received liver-directed treatment from June 2011 to August 2018 at Moffitt Cancer Center were retrospectively reviewed and analyzed to evaluate median OS, liver PFS, radiographic response and clinical and ...

[2] - by KL Zhu · 2019 · Cited by 11 — Therapeutic strategies include liver wedge resection or lobectomy, chemotherapy, radiotherapy and liver transplantation; however, the prognosis ...

[3] - by H Krzyston · 2020 · Cited by 9 — Liver-directed therapies included in this study were transarterial chemoembolization with doxorubicin eluting beads (DEB-TACE), yttrium-90 (Y90) ...

[4] - Tazemetostat (Tazverik). Tazemetostat works by targeting EZH2, a protein known to be involved in cancer cell growth.

[14] - For metastatic leiomyosarcoma, second-line treatment such as trabectedin, gemcitabine, or dacarbazine has led to an objective response in 4 to 10% of patients, with a median progression-free survival time of about 5 months and overall survival time between 14-16 months.

[15] - As a result, the treatment of advanced leiomyosarcoma is challenging. First-line therapy for metastatic and/or unresectable leiomyosarcoma includes anthracycline or gemcitabine based regimens, which provide a median progression-free survival time of about 5 months and overall survival time between 14-16 months.

Differential Diagnosis of Liver Leiomyosarcoma

Liver leiomyosarcoma, a rare and aggressive tumor, can be challenging to diagnose due to its similarity in appearance with other liver lesions. The differential diagnosis for liver leiomyosarcoma includes:

• Hepatocellular carcinoma: A primary liver cancer that can present with similar imaging characteristics as leiomyosarcoma.
• Fibrolamellar hepatocellular carcinoma: A rare subtype of hepatocellular carcinoma that can mimic the appearance of leiomyosarcoma on imaging studies.
• Metastatic non-hepatic leiomyosarcoma: Leiomyosarcomas originating from other parts of the body, such as the uterus or retroperitoneum, can metastasize to the liver and be mistaken for a primary liver tumor.
• Hemangioma: A benign liver tumor that is often hypervascular but more homogeneous in appearance compared to leiomyosarcoma.

Key Features to Distinguish Liver Leiomyosarcoma from Other Lesions

To accurately diagnose liver leiomyosarcoma, it is essential to consider the following features:

• Imaging characteristics: Liver leiomyosarcomas are often hypervascular and can have a heterogeneous appearance on imaging studies.
• Histopathological examination: A definitive diagnosis of leiomyosarcoma requires histopathological examination, which can reveal distinctive morphological features such as deep eosinophilic cytoplasm and blunt-ended nuclei.
• Clinical presentation: Liver leiomyosarcomas often present with nonspecific symptoms, but a thorough clinical evaluation can help rule out other potential causes.

References

[3] Primary hepatic leiomyosarcoma is a rare mesenchymal malignancy of the liver with an incidence of 6–16% of the primary hepatic mesenchymal malignancies. The diagnosis of this entity requires exclusion of a primary site namely genitourinary tract (especially gynaecologic tract in female patients), retroperitoneum, gastrointestinal tract ...

[12] Given the rarity of primary hepatic leiomyosarcoma, the possibility of metastasis from a distant primary tumour was entertained, for which reason positron emission tomography (PET)-CT was carried out. This demonstrated a sizeable focus of uptake within the hepatic tumour, occupying most of the right liver lobe ...

[14] the first differential diagnosis in all such cases and GIST must be ruled out before a confident diagnosis of leio-myosarcoma.Morphologically,GISTcanbeepithelioidor spindle cell type and the spindle cell type mimics leio-myosarcoma. The subtle morphological clues of leiomyo-sarcoma could be deep eosinophilic cytoplasm and blunt