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Angiomyomatous Hamartomas

In our articles, our goal is to provide educational information on any and all conditions that are related or associated with the lymph system and to lymphedema.

This page provides the most current information on angiomyomatous hamartomas, which is a rare type of tumor that originates from blood vessel tissue and forms most often in the inguinal (lower abdomen and groin) lymph nodes.

Pat O'Connor

June 25, 2009

Angiomyomatous Hamartomas

Definition

Angiomyomatous hamartoma is a primary vascular tumor primarily found in the inguinal and femoral lymph nodes characterized by the replacement of nodal tissue by smooth muscle cells and fibrous tissue in sclerotic lymphatic stroma. There has been 1 report of an angiomyomatous hamartoma of a cervical lymph node. (1)

Symptoms

There are basically two signs of a hamartomas are both are “lumps” that might arise in either the right groin area or lower abdominal areas.

Causes

Etiology is unknown

Complications

Possible complications include malignancy, lymphatic blockage, possible changes edematous stromal tissue, thickening of blood vessels and lymphatics.

Treatments

Hamartomas are generally treated by surgical removal. Other treatments include include the use of chemotherapy and radiation therapy.

Abstracts

Lymphoscintigraphy in angiomyomatous hamartomas and primary lower limb lymphedema

Clin Nucl Med. 2009 Jul

Bourgeois P, Dargent JL, Larsimont D, Munck D, Sales F, Boels M, De Valck C. Department of Nuclear Medicine, Institut Jules Bordet, Université Libre de Bruxelles, Brussels, Belgium. pierre.bourgeois@bordet.be

PURPOSE: Angiomyomatous hamartoma (AH) of the lymph node is a rare vascular benign disease of unknown etiology with a predisposition for the lymph nodes of the inguinal area. Only 18 cases have been described up to now in the literature and the disorder was reported to be associated with lymphedema or swelling of the ipsilateral limb in 4 patients. However, scintigraphic investigation of the lymphatic system in these patients was reported in only 2 cases.

MATERIAL AND METHODS: Five patients where the biopsy of inguinal nodes for suspected lymphadenitis led to the diagnosis of angiomyomatous hamartoma were investigated using lymphoscintigraphic techniques (1 girl aged 15; 1 boy aged 9 at the time of first biopsy and 11 at the time of the second one; and 3 men aged 30, 50, and 57). The operated limb was lymphedematous in 3 and 1 developed lymphedema after biopsy. The fifth patient developed a contralateral lymphedema after his second nodal biopsy.

RESULTS: In all cases, lymphoscintigraphic investigation of the limbs showed extensive lymph node abnormalities on the operated side and in 4 cases on the opposite side.

CONCLUSIONS: These observations support not only the hypothesis that lymphatic disturbance was involved in the pathogenesis of these tumors but also the proposition that lymphoscintigraphy should be performed in cases of inguinal lymphadenitis of unknown origin to diagnose the underlying situation of latent lymphedema.

Clinical Nuclear Medicine

Angiomyomatous hamartoma in a submandibular lymph node: a case report

Ear Nose Throat J. 2009 Mar

Barzilai G, Schindler Y, Cohen-Kerem R. Department of Otolaryngology-Head and Neck Surgery, Carmel Medical Center, Haifa, Israel. geva_b@hotmail.com

Angiomyomatous hamartomas have been found almost exclusively in the inguinal and femoral lymph nodes; few reports of these lesions in the head and neck region have been published. We present a case of angiomyomatous hamartoma in the submandibular area, a site that has not been previously reported in the literature. The mass was initially diagnosed as an enlarged lymph node. When it did not regress following broad-spectrum antibiotic treatment, the patient, a 51-year-old woman, underwent an excisional biopsy. Histopathology identified the mass as an angiomyomatous hamartoma. At follow-up 3 years and 9 months postoperatively, the patient exhibited no evidence of recurrence on physical examination and computed tomography. Even though angiomyomatous hamartoma of the head and neck is rare, we suggest that otolaryngologists include it in the differential diagnosis of head and neck masses.

PubMed

Angiomyomatous hamartoma of a popliteal lymph node: an unusual cause of posterior knee pain.

Ann Diagn Pathol. 2008 Oct

Mauro CS, McGough RL 3rd, Rao UN. Department of Orthopaedic Surgery, University of Pittsburgh, Pittsburgh, PA 15213, USA. maurocs@upmc.edu

Angiomyomatous hamartoma is a primary vascular tumor primarily found in the inguinal and femoral lymph nodes characterized by the replacement of nodal tissue by smooth muscle cells and fibrous tissue in sclerotic lymphatic stroma. There has been 1 report of an angiomyomatous hamartoma of a cervical lymph node, and this is the first reported case occurring in an extremity. We present a case of angiomyomatous hamartoma occurring in a single popliteal lymph node.

Elsevier

Angiomyomatous hamartoma and associated stromal lesions in the right inguinal lymph node: a case report

Pathol Int. 2000 Aug

Sakurai Y, Shoji M, Matsubara T, Imazu H, Hasegawa S, Ochiai M, Funabiki T, Mizoguchi Y, Kuroda M, Kasahara M. Department of Surgery and Pathology, Fujita Health University School of Medicine, Toyoake, Aichi, Japan. ysakurai@fujita-hu.ac.jp

KEYWORDS: angiomyomatous hamartoma • inguinal lymph node

Angiomyomatous hamartoma is a rare disease with a predisposition for the inguinal lymph nodes. A 51-year-old male patient visited a local hospital because of a right inguinal mass, measuring 3 x 4 cm in size, which was resected. The resected specimen showed irregularly distributed thick-walled vessels in the hilum, extending into the medulla and focally into the cortex of the node, eventually becoming more dispersed and associated with smooth muscle cells splaying into sclerotic stroma. These findings are compatible with an angiomyomatous hamartoma. Another tumor-like mass appeared shortly after the resection at the same location, but was not an angiomyomatous hamartoma, rather it was composed of edematous stromal tissue with proliferating smooth muscle cells. The stromal component included thick-walled blood vessels and lymphatics. Although it could not be determined whether these associated changes in the surrounding stroma are a cause or an effect of angiomyomatous hamartoma, they indicate the clinical difficulty in determining an appropriate area of resection and may provide clues to the pathogenesis of angiomyomatous hamartoma.

Wiley InterScience

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