Lymphangiomatosis With Splenic Involvement

Avigad, Sara; Jaffe, R. H.; Frand, Mira; Izhak, Yakov; Rotem, Y

doi:10.1001/jama.1976.03270210041022

Cited by 23 publications

(16 citation statements)

References 5 publications

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“…Solid areas may be seen, usually due to central scarring. 4,20 Lymphangiomas presenting as multiple nodules have similar-appearing satellite lesions surrounding a larger lesion, are separated by distinct residual splenic tissue, and can cause the spleen to be nodular and enlarged. 8 Cases of diffuse lymphangiomatosis can replace the spleen with expanding cysts that result in little remaining normal parenchyma (Figure 2).…”

Section: Gross Findingsmentioning

confidence: 99%

“…2,3 They were first described by Rodender in 1828; however, the first case involving the spleen was reported in 1885 by Frink. 4,5 Between 1939 and 2010, only 189 cases of splenic lymphangiomas were reported in the literature. 6 Therefore, splenic lymphangiomas are considered uncommon benign tumors, occurring mainly in childhood, with only a few cases reported in adults.…”

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confidence: 99%

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Splenic Lymphangioma

Ioannidis

Kahn

2015

Archives of Pathology &Amp; Laboratory Medicine

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Splenic lymphangioma is a rare malformation of the splenic lymphatic channels, mostly seen in children. It is characterized by the presence of cysts, resulting from increases in the size and number of thin-walled lymphatic vessels that are abnormally interconnected and dilated. The condition may be restricted to the spleen, but in most cases it involves multiple organs (systemic lymphangiomatosis). The clinical picture is variable; small lesions are often incidentally detected through imaging studies, while larger lesions can result in compression of organs, causing pain or rupture even after minor trauma. Therefore, splenic lymphangiomas should be considered in the differential diagnosis of splenomegaly or left upper quadrant pain even among adults and should be immediately treated with splenectomy; delay in the therapeutic intervention can lead to life-threatening complications.

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Section: Gross Findingsmentioning

confidence: 99%

mentioning

confidence: 99%

Splenic Lymphangioma

Ioannidis

Kahn

2015

Archives of Pathology &Amp; Laboratory Medicine

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“…They are usually found in the neck (75%) and axilla (20%) and less commonly encountered in the orbit, mediastinum, adrenal gland, kidney, bone, omentum, gastrointestinal tract, retroperitoneum, liver, spleen and pancreas 9–11. The first case involving the spleen was reported in 1885 byFrink 12. Between 1939 and 2010, only 189 cases lymphangiomas with splenic involvement were reported in the literature 13.…”

Section: Discussionmentioning

confidence: 99%

Isolated cystic lymphangiomatosis of spleen in an adult: a diagnostic conundrum

Kumar

Husain

et al. 2018

BMJ Case Reports

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Lymphangiomatosis is a rare developmental disorder characterised by diffuse proliferation of anastomosing lymphatic channels (lymphangiomas). It is believed to result from anomalous lymphatic development and usually presents in childhood. It typically occurs as a part of systemic lymphangiomatosis and isolated organ involvement is rare. Only nine cases of isolated cystic lymphangiomatosis of spleen have been reported between 1990 and 2010. Tuberculosis is a significant health problem in India and varied forms of this disease are seen in clinical practice. Isolated splenic tuberculosis, though a rare entity, has been described in the Indian population. We present a case of isolated splenic lymphangiomatosis in a 42-year-old woman that was initially misdiagnosed as splenic tuberculosis and was treated accordingly. Unresponsive to medical treatment, the patient underwent splenectomy and on histopathological examination, cystic lymphangiomatosis was diagnosed. The patient's symptoms resolved after surgery and she is doing well at a follow-up of 3 months.

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“…Lymphangioma is an uncommon primary splenic tumor that originates from the non-hematolymphoid tissue of the spleen's red pulp (1–4). These tumors may present in a variety of fashions, from incidental lesions noted on abdominal imaging to rapidly growing lesions resulting in splenic rupture (5–7).…”

mentioning

confidence: 99%

“…These tumors may present in a variety of fashions, from incidental lesions noted on abdominal imaging to rapidly growing lesions resulting in splenic rupture (5–7). Moreover, lymphangiomatosis may occur in children when there is mediastinal, axillary, and neck involvement by lymphangioma (4). In terms of imaging characteristics, the literature suggests that splenic lymphangiomas are generally cystic lesions that are hypoechoic or anechoic on ultrasound with possible septations (2, 8).…”

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confidence: 99%

Unusual imaging profile of a solitary splenic lymphangioma

Eghtedari¹,

Sicklick²,

Kono³

et al. 2012

Acta Radiologica Short Reports

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Splenic lymphangioma is a rare, benign lesion of the spleen that is characterized microscopically by a proliferation of thin-walled vascular channels filled by proteinacious material. Based on microscopic features, three types of lymphangiomas are described as simple capillary, cavernous, and cystic; however, the distinction between these types is not uniformly accepted. The cystic type is the most common type and has well described imaging characteristics. However, little is known about the less common cavernous and capillary forms of lymphangioma. Herein, we present a case of a patient with two synchronous colon cancers that presented with a concomitant splenic mass that was initially suspected to be metastatic disease. Despite the use of multiple imaging modalities including computed tomography (CT), magnetic resonance imaging (MRI), nuclear imaging, and contrast-enhanced ultrasound, the final diagnosis of cavernous lymphangioma was made following core needle biopsy of the lesion. A thorough review of literature and this case now demonstrate the difficulty in differentiating atypical forms of splenic lymphangiomas from other benign lesions such as hemangioma.

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Lymphangiomatosis With Splenic Involvement

Cited by 23 publications

References 5 publications

Splenic Lymphangioma

Splenic Lymphangioma

Isolated cystic lymphangiomatosis of spleen in an adult: a diagnostic conundrum

Unusual imaging profile of a solitary splenic lymphangioma

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