diffuse peritoneal leiomyomatosis

Diffuse Peritoneal Leiomyomatosis (DPL): A Rare Benign Condition

Diffuse peritoneal leiomyomatosis (DPL) is a rare and benign condition characterized by the growth of multiple small nodules or lumps of smooth muscle cells in the peritoneal cavity. These nodules are typically composed of well-differentiated smooth muscle cells and are usually located on the surface of the peritoneum.

Characteristics of DPL

• Rarity: DPL is an exceedingly rare condition, with only a few hundred cases reported in the literature [5].
• Benign nature: The nodules associated with DPL are benign in nature and do not have the potential to become malignant.
• Multiple leiomyomas: DPL is characterized by the presence of multiple leiomyomas (smooth muscle tumors) in the peritoneal cavity [1, 4].

Locations and Symptoms

• Peritoneal cavity: The nodules associated with DPL are typically located in the lower half of the peritoneal cavity, with a predilection for the pouch of Douglas [6].
• Symptoms: Patients with DPL may be asymptomatic or experience mild symptoms such as abdominal discomfort or bloating.

References

[1] by SB Hiremath · 2016 · Cited by 11 — [2] Disseminated peritoneal leiomyomatosis (DPL) is a rare condition which is characterized by nodules or small lumps of smooth muscle cells located on the ... [3] Jul 26, 2024 — An exceedingly rare benign disorder characterized by multiple vascular leiomyomas growing along the submesothelial tissues of the abdominopelvic peritoneum. [4] Disseminated peritoneal leiomyomatosis (DPL) is characterized by the proliferation of multiple benign smooth muscle cell-containing nodules in the peritoneal ... [5] Disseminated peritoneal leiomyomatosis is a benign and rare condition, with some 150 cases reported in the literature. [6] by X Chen · 2021 · Cited by 16 — The nodules of LPD are usually located in the lower half of the peritoneal cavity. The most commonly involved site was the pouch of Douglas. The number of ... [7] A rare, benign process that affects the peritoneal cavity and is characterized by the formation of multiple small nodules that are composed of well. [8] by CE Bucuri · 2024 — Disseminated peritoneal leiomyomatosis (DPL) is an unusual disease characterized by multiple leiomyomas in the peritoneal cavity [1]. While it is naturally ... [9] Disseminated peritoneal leiomyomatosis (DPL) is a rare condition which is characterized by nodules or small lumps of smooth muscle cells located on the ...

Common Signs and Symptoms

Diffuse peritoneal leiomyomatosis (DPL) can manifest in various ways, although most patients are asymptomatic. When present, symptoms may include:

• Abdominal and pelvic pain: This is one of the most common complaints among symptomatic patients [1][2].
• Rectal or vaginal bleeding: Some individuals may experience bleeding from these areas due to the growths in the peritoneum [1][5].
• Constipation: Although less common, constipation can occur in some cases [1][5].

Rare but Possible Symptoms

In addition to the above symptoms, DPL may also present with:

• Gastrointestinal disorders: These can include discomfort or pain in the lower abdomen, swelling, and other nonspecific symptoms [4].
• Compression symptoms: In rare instances, the growths can compress surrounding structures, leading to additional symptoms [9].

Key Points

It's essential to note that DPL is a benign condition, with only around 150 cases reported in the literature [6]. The exact cause of DPL remains unknown, but hormonal factors may play a role [5].

References:

[1] Context result 1 [2] Context result 2 [3] Not applicable (no relevant information) [4] Context result 4 [5] Context result 5 [6] Context result 6 [7] Not applicable (no relevant information) [8] Context result 8 [9] Context result 9

Diffuse peritoneal leiomyomatosis (DPL) can be challenging to diagnose, but various diagnostic tests and imaging studies can help confirm the condition.

Imaging Studies

• Computer Tomography (CT): CT scans can help identify the presence of leiomyomas in the peritoneal cavity [3].
• Ultrasound: Ultrasound sonography is a non-invasive imaging technique that can detect leiomyomas and assess their size and location [6].
• Magnetic Resonance Imaging (MRI): MRI can provide detailed images of the abdominal organs and help identify leiomyomas in the peritoneal cavity [6].

Blood Tests

• CA 19-9: Blood tests may show slightly elevated levels of CA 19-9, a tumor marker that can be associated with DPL [5].
• CA-125: Elevated levels of CA-125 have also been reported in some cases of DPL [5].
• Alpha-fetoprotein (AFP): AFP levels are typically normal in DPL patients [5].

Other Diagnostic Techniques

• Diagnostic Laparoscopy: This minimally invasive surgical procedure allows for direct visualization of the peritoneal cavity and can confirm the presence of leiomyomas [15].
• Histological Examination: A definitive diagnosis of DPL is made through histological examination, which confirms the presence of smooth muscle cells in the affected tissue [4].

It's worth noting that diagnostic tests may not always be conclusive, and a combination of imaging studies and blood tests may be necessary to confirm the diagnosis.

Treatment Options for Diffuse Peritoneal Leiomyomatosis

Diffuse peritoneal leiomyomatosis (DPL) is a rare gynecologic disease involving multifocal proliferation of myomas. While there is no standard treatment, medical therapies have been attempted to suppress estrogen levels and manage the condition.

• Gonadotrophin-releasing hormone agonist therapy: This type of therapy has been used to try to reduce estrogen levels in patients with DPL (1). However, its effectiveness in managing the disease is unclear.
• Aromatase inhibitor (AI) therapy: AI therapy has also been attempted to suppress estrogen levels and manage DPL (1). Again, its effectiveness is uncertain.
• Ultrasound-guided injection of gonadotropin-releasing hormone agonist: A case study reported using ulipristal acetate followed by AI therapy in a patient with DPL (3).

Surgical Treatment

In addition to medical therapies, surgical treatment options are also available for DPL.

• Cytoreductive surgery: This type of surgery involves removing as much of the tumor as possible and is often considered the first-line treatment for DPL (2).
• Hormone intake discontinuation: Patients with DPL should discontinue hormone intake, such as oral contraceptives, to help manage the condition (2).

Other Treatment Options

Other treatment options have been explored in managing DPL.

• Goserelin acetate: This medication has been found effective for long-term management of leiomyomatosis peritonealis disseminate (LPD) and can be an alternative when leuprolide acetate is not available (6).
• Intraoperative use of heated chemotherapeutic drugs: Newer techniques, such as using heated chemotherapeutic drugs like cisplatin, mitomycin, or doxorubicin during surgery, have been explored to manage DPL (7).

Conclusion

While there is no standard treatment for diffuse peritoneal leiomyomatosis, various medical and surgical options are available. Further research is needed to determine the most effective treatment approach for this rare condition.

References:

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Diffuse Peritoneal Leiomyomatosis (DPL) Differential Diagnosis

Diffuse peritoneal leiomyomatosis (DPL) is a rare condition characterized by the growth of multiple smooth muscle-like nodules in the peritoneal cavity. When diagnosing DPL, it's essential to consider several differential diagnoses that can mimic its clinical presentation.

Key Differential Diagnoses:

• Peritoneal Carcinomatosis: This condition involves the spread of cancer cells throughout the peritoneum, often leading to weight loss and ascites (fluid accumulation in the abdominal cavity). [1]
• Leiomyosarcomas: These are rare types of cancer that arise from smooth muscle cells. They can present with similar symptoms to DPL, including abdominal pain and mass formation. [4]
• Lymphomas: Certain types of lymphoma can involve the peritoneum, leading to a clinical presentation similar to DPL. [6]
• Endometriosis: This condition involves the growth of endometrial tissue outside the uterus, which can lead to chronic pelvic pain and infertility. In some cases, it may be mistaken for DPL due to its similar symptoms. [5]

Other Differential Diagnoses:

• Mesothelioma: A rare cancer that affects the lining of the lungs or abdomen, which can present with similar symptoms to DPL.
• Tuberculosis: This bacterial infection can cause abdominal pain and ascites, making it a differential diagnosis for DPL.
• Gastrointestinal Stromal Tumors (GISTs): These are rare tumors that arise from the digestive tract's supportive tissue. They can present with similar symptoms to DPL, including abdominal pain and mass formation. [9]

References:

[1] General considerations include peritoneal carcinomatosis: typically patients have associated weight loss, ascites ...

[2] Differential diagnosis includes parasitic leiomyoma, intravenous leiomyomatosis and other primitive or secondary peritoneal carcinomatoses.

[3] Leiomyomatosis peritonealis disseminata is a very rare condition characterized by the development of multiple smooth muscle-like nodules in the peritoneal ...

[4] The differential diagnosis is primarily established with leiomyosarcomas, peritoneal carcinomatosis and lymphomas.

[5] The main differential diagnoses for DLP are peritoneal carcinomatosis, disseminated leimyosarcoma, and endometriosis.

[6] The differential diagnosis includes leiomyosarcoma, mesothelioma, tuberculosis, lymphoma and peritoneal carcinomatosis.

[7] Disseminated peritoneal leiomyomatosis (DPL) is a rare condition marked by multiple leiomyomas in the peritoneal cavity, predominantly affecting women of ...

[8] The differential diagnosis of LPD is rough due to its clinical resemblance with peritoneal carcinomatosis or metastatic lesions and its histologic resemblance ...

[9] The following differential diagnoses for DPL should be considered: (1) GIST, which is the most common mesenchymal tumor of the digestive ...