Massive splenomegaly caused by cavernous hemangiomas associated with Klippel–Trenaunay syndrome: report of a case

Misawa, Takeyuki; Shiba, Hiroaki; Fujiwara, Yuki; Futagawa, Yasuro; Harada, Taishi; Ikegami, Machiko; Yanaga, Katsuhiko

doi:10.1007/s00595-013-0779-y

Cited by 4 publications

(3 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…The tumors were heterogeneous and poorly demarcated. (10) Although we initially considered hemangioma, large splenic tumors sometimes mimic angioblastoma (11). In cases of splenic angiosarcoma, surgery is the only curative treatment, and hence, the chance to resect such tumors must not be missed.…”

Section: Discussionmentioning

confidence: 99%

Splenic hemangiomas with different imaging findings: two laparoscopically resected incidentalomas

Hiyama¹,

Koyanagi²,

Fukui³

et al. 2021

Preprint

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 99%

Splenic hemangiomas with different imaging findings: two laparoscopically resected incidentalomas

Hiyama¹,

Koyanagi²,

Fukui³

et al. 2021

Preprint

View full text Add to dashboard Cite

show abstract

“…In PROS, ectatic portomesenteric veins can occur and lead to thrombosis and portal hypertension (Alomari et al, ). Splenomegaly with multiple VMs has been documented in KTS (Misawa et al, ), which is characterized by a triad of port‐wine stain, varicose veins, and bony and soft tissue overgrowth involving an extremity.…”

Section: Discussionmentioning

confidence: 99%

Characterization of the hepatosplenic and portal venous findings in patients with Proteus syndrome

Takyar

Khattar

Ling

et al. 2018

American J of Med Genetics Pt A

View full text Add to dashboard Cite

Proteus syndrome (PS) is a rare disorder caused by a mosaic AKT1 variant that comprises patchy overgrowth of tissues derived from all three germinal layers affecting multiple viscera. We sought to delineate the extent of hepatoportal manifestations in patients with PS. We identified patients with PS who had abdominal imaging from 1989 to 2015 in a natural history study.Imaging was characterized for evidence of focal findings in the liver, spleen, and portal vasculature and for organomegaly. Relevant clinical and laboratory data were compared among those with or without organomegaly. Abdominal imaging was available on 38 patients including 20 who had serial studies. Nine patients had focal hepatic lesions including vascular malformations (VMs). Focal splenic abnormalities were noted in seven patients. Patients without cutaneous VMs did not have visceral VMs. Nine patients had splenomegaly, 12 had portal vein dilation, and 4 had hepatomegaly. There was a weak correlation of portal vein dilation to spleen height ratio (r 2 = 0.18, p < .05). On laboratory evaluation, hepatic function was normal but there was thrombocytopenia in those with splenomegaly; platelet counts were 179 AE 87K/μL compared to those with normal spleen size at 253 AE 57K/μL (p < .05). Overall, focal hepatosplenic abnormalities occurred in 11 of 38 (29%) patients with PS. Splenomegaly and portal venous dilation were both found in 8 of 38 (21%) patients; however, other than relative thrombocytopenia, there was no evidence of portal hypertension. Although the AKT1-E17K somatic variant is a suspected oncogene, there were no malignant lesions identified in this study. K E Y W O R D SAKT1 mutation, overgrowth syndromes, Proteus syndrome, vascular malformations

show abstract

“…The signal characteristics of the lesions were discussed in Part 1 of this paper. Additional common imaging findings of KTS include bony, soft tissue, and craniofacial hypertrophy, brain aneurysms and malformations, gastrointestinal and genitourinary vascular anomalies, and splenic hemangiomas 25,27–29 …”

Section: Laser Ablationmentioning

confidence: 99%

An overview of interventional radiology techniques for the diagnosis and management of vascular anomalies: Part 2

Scollan

Azimov

Garzon

et al. 2023

Pediatric Dermatology

View full text Add to dashboard Cite

Minimally invasive percutaneous and endovascular strategies performed by interventional radiologists have become the mainstays of treatment for vascular anomalies with improved outcomes, decreased complication rates, and less morbidity. The aim of this article is to introduce physicians who care for patients with vascular anomalies to state‐of‐the‐art advancements in interventional radiology for diagnosis and treatment. Part 2 of this review discusses embolization, endovenous laser ablation, and image‐guided percutaneous biopsy. Please see Part 1 for a discussion of sclerotherapy and cryoablation. Select vascular anomalies will be discussed as examples to highlight IR diagnostic and/or treatment techniques.

show abstract

Massive splenomegaly caused by cavernous hemangiomas associated with Klippel–Trenaunay syndrome: report of a case

Cited by 4 publications

References 10 publications

Splenic hemangiomas with different imaging findings: two laparoscopically resected incidentalomas

Splenic hemangiomas with different imaging findings: two laparoscopically resected incidentalomas

Characterization of the hepatosplenic and portal venous findings in patients with Proteus syndrome

An overview of interventional radiology techniques for the diagnosis and management of vascular anomalies: Part 2

Contact Info

Product

Resources

About