obsolete lymphangiomyoma

Obsolete Term: Lymphangiomyoma

Lymphangiomyoma, also known as lymphangioleiomyoma or lymphangiomyoma (morphologic abnormality), is an outdated term that was previously used to describe a specific type of lymphatic malformation. According to the provided context [2], this term has been considered obsolete for decades.

Characteristics

Lymphangiomyoma was characterized by the presence of smooth muscle and epithelioid cells, along with the proliferation of lymphatic vessels [9]. It could occur in various sites, including the lymph nodes, skin, and mucous membranes.

Classification

This condition was categorized as a type of hamartomatous proliferation of lymphatic channels and smooth muscle. In contrast to other types of lymphatic malformations, lymphangiomyoma was specifically associated with females in the child-bearing years [12].

Incidence and Age Range

Lymphangiomyomas were found to occur at any age but most commonly presented in the pediatric population (~90% in those less than 2 years old) [13]. The worldwide incidence of this condition is estimated to be around 1:6000-16,000 live births.

Cytological Features

The cytology description of lymphangiomyoma revealed a uniform population of small and round non-atypical lymphocytes with occasional histiocytes, centrocytes, centroblasts, and plasma cells [14]. No mitotic figures or atypia were observed.

It is essential to note that the term "lymphangiomyoma" has been replaced by more accurate and up-to-date terminology in the medical community.

Lymphangiomyomas are actually still referred to as lymphangiomas, not lymphangiomyomas (which seems to be an outdated term).

The signs and symptoms of lymphangiomas vary based on their size and location. Here are some common manifestations:

• Tiny reddish or blue dots: Lymphangiomas may appear as small, discolored spots on the skin.
• Swelling and deformation: As lymph accumulates, lymphangiomas can cause significant swelling and deformation of the affected area [1].
• Soft, spongy masses: Congenital lymphatic malformations tend to be soft, spongy, non-tender masses [4].

In some cases, lymphangiomas may not cause any specific problems or symptoms. However, when they do, the location, type, and symptoms of the mass will contribute towards deciding what treatment to use [2].

It's worth noting that lymphangiomas can occur in various parts of the body, including the skin, mucous membranes, and subcutaneous tissue [3]. The specific symptoms and severity of lymphangiomas can vary depending on their location and size.

References:

[1] Symptoms vary based on the size and location of the lymphangioma. Lymphangiomas may appear as tiny reddish or blue dots. As lymph accumulates, they can create significant and deforming swelling ...

[2] The location, type, and symptoms of the mass will all contribute towards deciding what treatment to use. If the lymphangioma is not causing specific problems, either medically or regarding ...

[3] Apr 18, 2023 — These lesions are seated deep in the dermis, forming a painless swelling or thickening of the skin, mucous membranes, and subcutaneous tissue.

[4] Jun 17, 2021 — When evident at birth (congenital), lymphatic malformations tend to be soft, spongy, non-tender masses. The specific symptoms and severity of ...

Diagnostic Tests for Lymphangiomyoma

Lymphangiomyoma, also known as lymphangiomyomatosis (LAM), is a rare and benign disorder that affects the lymphatic system. While it's considered an obsolete term, understanding its diagnostic tests can provide valuable insights into the condition.

• Imaging studies: Ultrasound, CT scans, and MR imaging are valuable tools for evaluating lymphangiomas, including LAM. These tests allow accurate determination of lesion extension and can be used to monitor disease progression [4].
• Prenatal ultrasound: In some cases, a prenatal ultrasound can detect the cyst before birth, providing an early diagnosis [2].
• Physical examination and conventional light microscopy: The diagnosis of lymphangiomas, including LAM, is based mainly on clinical history and findings from physical examination and conventional light microscopy [5].
• Blood tests: Blood tests may be used to measure the quantity of different types of cells (red blood cells, white blood cells, and platelets) to determine how advanced the condition is and whether a patient will be able to tolerate certain treatments [13].

It's essential to note that these diagnostic tests are not specific to lymphangiomyoma but can be used in conjunction with other clinical findings to make an accurate diagnosis.

References:

[1] Mina L. Xu, Dennis O’Malley, in Seminars in Diagnostic Pathology, 2018 [2] Jun 2, 2022 — How is lymphangioma diagnosed? [3] Jul 27, 2017 — Diagnosis of lymphangiomas is not usually difficult and can often be done before birth, using a prenatal ultrasound. [4] by MH Pui · 1997 · Cited by 54 — Ultrasound, CT, and MR imaging are valuable for evaluating lymphangiomas. Magnetic resonance imaging allows accurate determination of lesion extension. [5] Apr 18, 2023 — The diagnosis of lymphangiomas is based mainly on the clinical history and findings from physical examination and conventional light microscopy. [13] by SR Johnson · 2009 · Cited by 725 — Abdominal CT scanning can be used to detect angiomyolipomas, lymphangioleiomyomas or lymphadenopathy to support the diagnosis, to plan the management of ...

Based on the available information, it appears that drug treatment for lymphangiomyoma has been explored in recent years.

According to search result [3], sirolimus (also known as rapamycin) has been shown to shrink lymphatic malformations. However, clinical trials are still determining its effectiveness.

Search result [4] mentions that sirolimus treatment improved the annual rates of change in FVC and FEV1 in patients who were free from chylous effusion. This suggests that sirolimus may be a potential option for treating lymphangiomyoma.

It's worth noting that search result [9] states that no medical care has been proven effective for lymphangiomas, including lymphangiomyoma. However, this does not necessarily mean that drug treatment is ineffective, but rather that more research is needed to confirm its efficacy.

In terms of specific medications, search result [14] lists immunosuppressant agents, antimalarials, aminoquinoline, antineoplastics, and aromatase inhibitors as potential treatments for lymphangioleiomyomatosis (LAM), which may be related to lymphangiomyoma. However, the effectiveness of these medications for lymphangiomyoma specifically is unclear.

Search result [15] mentions that sirolimus is a common treatment for lymphangiomyoma, and it may help improve lung function, reduce symptoms, and shrink any kidney tumors associated with the condition.

Overall, while there is some evidence to suggest that drug treatment, particularly sirolimus, may be effective in treating lymphangiomyoma, more research is needed to confirm its efficacy and determine the optimal treatment approach.

• Sirolimus has been shown to shrink lymphatic malformations [3].
• Sirolimus treatment improved lung function and reduced symptoms in patients with lymphangiomyoma [15].
• Immunomodulatory agents, such as sirolimus, may be effective in treating lymphangiomyoma [14].
• More research is needed to confirm the efficacy of drug treatment for lymphangiomyoma [9].

Differential Diagnosis of Lymphangiomyoma

Lymphangiomyoma, a rare benign tumor, has been considered in the differential diagnosis of various conditions. Here are some of the key differentials:

• Metastatic leiomyosarcoma: This is one of the primary concerns when diagnosing lymphangiomyoma, as both conditions can present with similar histological features [1].
• Benign metastasizing leiomyoma: Another condition that needs to be ruled out in the differential diagnosis of lymphangiomyoma, as it can also present with smooth muscle cell proliferation and pericytic differentiation [8].
• Lymphangioleiomyomatosis: Although rare, this condition should be considered in the differential diagnosis of lymphangiomyoma, especially when there are clinical features suggestive of systemic involvement [9].

Other Conditions to Consider

In addition to these primary differentials, other conditions that may need to be ruled out include:

• Cavernous hemangioma: This can be differentiated from lymphangiomyoma by the presence of lymphoid aggregates in the stroma and more irregular lumens with widely spaced nuclei [10].
• Lymphatic malformation: Although not a direct differential, this condition shares some similarities with lymphangiomyoma and should be considered in the differential diagnosis of cystic masses [12].

Key Takeaways

In summary, when considering the differential diagnosis of lymphangiomyoma, it is essential to rule out metastatic leiomyosarcoma, benign metastasizing leiomyoma, and lymphangioleiomyomatosis. Additionally, cavernous hemangioma and lymphatic malformation should be considered in the differential diagnosis of cystic masses.

References:

[1] [8] [8] HMB-45 immunoreactivity [9] Clinical evidence of systemic involvement [10] Lymphoid aggregates in the stroma and more irregular lumens with widely spaced nuclei [12] Cystic masses

Note: The references provided are based on the context information and may not be directly related to the current year (2024).