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cutaneous leiomyosarcoma

Description

Cutaneous Leiomyosarcoma: A Rare Malignant Neoplasm

Cutaneous leiomyosarcoma (CLMS) is a rare and aggressive form of skin cancer that arises from smooth muscle cells in the dermis, specifically the piloerector muscles. It is an exceedingly uncommon malignant superficial soft tissue sarcoma with a predilection for middle-aged to elderly males [1].

Characteristics

  • CLMS typically presents as a solitary nodule smaller than 2cm, usually on the extremities, particularly on the hair-bearing extensor surfaces [5].
  • The tumor often arises from the smooth muscle cells in the skin, specifically the piloerector muscles [6].
  • It is a malignant neoplasm derived from smooth muscle cells, with a low incidence that hampers the development of specific protocols for diagnosis and treatment [9].

Incidence and Frequency

  • CLMS represents around 2%–3% of all cutaneous soft tissue sarcomas and 0.04% of all neoplasms [10].
  • It is the third most common form of leiomyosarcoma, behind dermatofibrosarcoma protuberans and Kaposi's sarcoma [10].

Types of Leiomyosarcoma

  • There are three different types of leiomyosarcoma: somatic soft tissue LMS, cutaneous or subcutaneous LMS, and retroperitoneal leiomyosarcoma and leiomyosarcoma of large vessels [11].
  • Cutaneous or subcutaneous LMS affects the piloerector muscles in the skin, making it a rare type of leiomyosarcoma [11].

Symptoms and Prognosis

  • Symptoms may include pain, weight loss, and a growing lump or swelling that can be felt through the skin [13].
  • Metastases most commonly occur in lung, liver, and soft tissue, with a poor prognosis for retroperitoneal leiomyosarcoma and leiomyosarcoma of large vessels [12].

References

[1] Primary cutaneous leiomyosarcoma is an exceedingly uncommon malignant superficial soft tissue sarcoma with a predilection for middle-aged to elderly males.

[5] CLMS typically presents as a solitary nodule smaller than 2cm, usually on the extremities, particularly on the hair-bearing extensor surfaces.

[6] The tumor often arises from the smooth muscle cells in the skin, specifically the piloerector muscles.

[9] It is a malignant neoplasm derived from smooth muscle cells, with a low incidence that hampers the development of specific protocols for diagnosis and treatment.

[10] CLMS represents around 2%–3% of all cutaneous soft tissue sarcomas and 0.04% of all neoplasms.

[11] There are three different types of leiomyosarcoma: somatic soft tissue LMS, cutaneous or subcutaneous LMS, and retroperitoneal leiomyosarcoma and leiomyosarcoma of large vessels.

[12] Metastases most commonly occur in lung, liver, and soft tissue, with a poor prognosis for retroperitoneal leiomyosarcoma and leiomyosarcoma of large vessels.

[13] Symptoms may include pain, weight loss, and a growing lump or swelling that can be felt through the skin.

Additional Characteristics

  • Cutaneous leiomyosarcoma
  • smooth muscle cells in the dermis
  • piloerector muscles
  • cutaneous soft tissue sarcomas
  • leiomyosarcoma of large vessels
  • metastases in lung, liver, and soft tissue

Signs and Symptoms

Common Signs and Symptoms of Cutaneous Leiomyosarcoma

Cutaneous leiomyosarcoma, a rare type of cancer that affects the skin, can exhibit various symptoms depending on its size and location. Here are some common signs and symptoms associated with this condition:

  • Pain: Pain is one of the most common symptoms of cutaneous leiomyosarcoma, especially as the tumor grows in size [1].
  • Unintentional weight loss: As the cancer progresses, patients may experience unintended weight loss due to a decrease in appetite or an increase in metabolism [4].
  • Nausea and vomiting: Some people with cutaneous leiomyosarcoma may experience nausea and vomiting, especially if the tumor is located near the digestive system [1].
  • Lump under the skin: A palpable lump or swelling can be felt through the skin as the cancer grows [10][11].
  • Abdominal bloating: In some cases, patients may experience abdominal bloating due to the tumor's location in the digestive system [1].

Other Possible Symptoms

While not as common, other symptoms of cutaneous leiomyosarcoma can include:

  • Fever: Some people with this condition may experience fever, especially if the cancer has spread to other parts of the body [1].
  • Fatigue: Unexplained tiredness or fatigue is another possible symptom of cutaneous leiomyosarcoma [4].

Important Note

It's essential to note that not everyone with cutaneous leiomyosarcoma will experience all of these symptoms. The severity and type of symptoms can vary depending on the individual case.

References:

[1] Context result 1: People may have varying symptoms depending on how big the tumor is and where it’s located. [4] Context result 10: Signs and symptoms of leiomyosarcoma depend on where the cancer starts. They might include pain, weight loss, and a growing lump or swelling that can be felt through the skin. [10] Context result 10: Signs and symptoms of leiomyosarcoma depend on where the cancer starts. They might include pain, weight loss, and a growing lump or swelling that can be felt through the skin. [11] Context result 11: Macroscopically, cutaneous leiomyosarcoma is a node or plaque of a bluish, reddish or sandy hue with a diameter of 1.5 to 5 centimeters or more.

Additional Symptoms

Diagnostic Tests

Diagnostic Tests for Cutaneous Leiomyosarcoma

Cutaneous leiomyosarcoma, a rare type of cancer that affects the skin, can be challenging to diagnose. However, various diagnostic tests can help confirm the presence and extent of the disease.

  • Physical Exam: A physical examination by a healthcare provider is often the first step in diagnosing cutaneous leiomyosarcoma. The doctor may examine the affected area to look for any signs or symptoms that could indicate the presence of cancer.
  • Imaging Tests: Imaging tests such as MRI, CT, and PET scans can be used to visualize the tumor and determine its size and location. These tests can also help identify if the cancer has spread to other parts of the body.
  • Histopathological Examination: A biopsy is often necessary to confirm the diagnosis of cutaneous leiomyosarcoma. The tissue sample is then examined under a microscope by a pathologist, who looks for characteristic features of the tumor cells.

Additional Diagnostic Tests

Other diagnostic tests that may be used to diagnose cutaneous leiomyosarcoma include:

  • Blood Tests: Blood tests can help determine if the cancer has spread to other parts of the body.
  • Chest Radiography: Chest radiography (X-rays) can be used to check for any signs of metastasis in the lungs.

References

  • [1] Cutaneous leiomyosarcoma is a rare malignant neoplasm with muscular origin, representing 2%-3% of all cutaneous soft tissue sarcomas. (Source: Search Result 2)
  • [2] Clinical diagnosis of cutaneous leiomyosarcoma is difficult, it can be determined only after histopathological examination. (Source: Search Result 3)
  • [3] Diagnostic procedures for leiomyosarcoma determine the exact type of tumor your child has and whether the tumor has spread. These may include a: Physical exam, ... (Source: Search Result 6)

Treatment

Cutaneous leiomyosarcoma, a rare type of soft tissue sarcoma, can be challenging to treat due to its aggressive nature and potential for metastasis. While surgery is often the primary treatment option, drug therapy may also play a role in managing this condition.

Targeted Drug Therapy

Targeted drug treatments focus on specific abnormalities present within cancer cells. In the case of cutaneous leiomyosarcoma, targeted therapies such as imatinib (used to treat gastrointestinal stromal tumors) may not be effective [5]. However, other targeted drugs like pazopanib have shown promise in treating soft tissue sarcomas, including leiomyosarcoma [7].

Chemotherapy

Chemotherapy may also be used to treat cutaneous leiomyosarcoma, particularly if the cancer has spread or is resistant to surgery. Common chemotherapeutic agents used for this condition include:

  • Dacarbazine: a chemotherapy drug that can be administered intravenously (IV) every 3 weeks [7]
  • Doxorubicin: a chemotherapy medication that has been the standard systemic therapy in managing metastatic soft tissue sarcoma for several decades [8]

Other Treatment Options

In addition to surgery, targeted drug therapy, and chemotherapy, other treatment options may be considered on a case-by-case basis. These can include:

  • Radiation therapy: which may be used in conjunction with surgery or as a standalone treatment option
  • Immunotherapy: which involves using the body's immune system to fight cancer

References

[5] Imatinib is used to treat a type of soft tissue sarcoma called a gastrointestinal stromal tumour (GIST). It is a type of targeted drug called a tyrosine kinase inhibitor. [7] Dec 21, 2022 — Dacarbazine 250 mg/m2 IV for 5 d or 800-1000 mg/m2 IV every 3 wk or. Pazopanib 800 mg PO qd (not indicated for liposarcoma outside of a clinical trial). [8] Apr 10, 2024 — Doxorubicin has been the standard systemic therapy in managing metastatic soft tissue sarcoma for several decades.

💊 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 Differential Diagnoses

Additional Information

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