Hamartoma Lymphangiomatosis – Causes, Symptoms, and Treatment

Hamartoma is a malformation of benign (non-cancerous) tissue growth consisting of an abnormal mixture of cells and tissue found in certain areas of the body. Hamartoma comes from 2 words: hamartia and oma, in Greek hamartia means error, defect and oma denotes a tumor or neoplasm.

This is considered an error in cell development and can occur in a number of areas in the body. Non-neoplastic masses can also arise this way. Therefore, misdiagnosis can occur, and excessive therapy due to misdiagnosis can also cause this.

The following are some examples of hamartomas, including:

  • Hemangiomas and other vascular tumors that are not neoplasms.
  • Peutz-Jeghers intestinal polyps.
  • Large intestine polyps.
  • Bronchial Hamartoma.
  • Melanocytic nevi.
  • Neurofibromatosis in von Recklinghausen’s disease.
  • Neuroepithelial cells in tuberous sclerosis.
  • Hamartoma of the hypothalamus and tuber cinereum.
  • Various bone hamartomas.

Meanwhile, lymphangiomatosis is a condition that affects lymph nodes, this is usually found accidentally such as broken bones for no apparent reason, enlargement of soft tissue, or the accumulation of tissue.

This condition is a very rare condition, and is usually only found in patients aged under five. The condition of lymphangiomatosis can be accompanied by hemangiomatosis, where in addition to lymph nodes, abnormalities also occur in blood vessels. To note, this disease does not have a tendency to be inherited in the family.

Symptoms of Hamartoma Lymphangiomatosis

Until now, the prevalence of lymphangiomatosis hamartoma is still unknown because the diagnosis is often illegible or discovered accidentally. Patients with this condition also often do not complain of anything, but there are also those who complain of pain, swelling in the affected body part, and fractures occur without apparent cause (no impact whatsoever).

Diagnosis of Hamartoma Lymphangiomatosis

  • X-ray examination: Bone with lymphangiomatosis will show a vertical line which means there is thickening of the trabeculae from the bone, in addition, a picture can be found like a honeycomb (honeycomb apperance).
  • CT scan: This examination shows the appearance of rough bones.
  • MRI examination, both T1 and T2: MRI is a standard criterion for investigating hemangiomas and lymphangiomas.
  • Angiogram examination: To see if there is a bulge in a blood vessel.
  • Radionuclide bone scan: Although it does not always produce positive results, this examination can find several lesions.
  • Lymphography: This examination is performed to show abnormal and dilated lymph vessels filling bone lesions.

Handling of lymphomaomatosis Hamartoma

Someone who experiences lymphangiomatosis hamartoma, the lesion can undergo regression so that it needs surgery. When this is done it will usually involve curettage, grafting, and irradiation. Surgery may be needed to treat pathological fractures or bone deformity.

Because hemangiomatosis and lymphangiomatosis are rare, each patient must be evaluated and treated individually with their own prognosis.

Bone hemangioma. This radiological picture of the spine shows the presence of trabecular vertical thickening.

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