Presentation
This report presents the case of a nulliparous premenopausal woman with no other contributory history who presented with DPL 3 years after UAE. The presentation of this patient suggests the potential for a causal relationship between UAE and DPL. [ncbi.nlm.nih.gov]
Entire Body System
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Swelling
Clinical examination only showed right lower limb swelling. Routine laboratory examination revealed no abnormal results. Abdominal ultrasonography identified uterine leiomyoma and soft tissue masses. [ncbi.nlm.nih.gov]
A 52-year-old female with no significant past medical history presented to her primary care physician with bilateral lower extremity swelling and lower extremity venous varicosities, both of which had worsened during the previous 2 months. [healio.com]
It is common in young women and is manifested by abdominal pain, tenderness or swelling. Primary peritoneal carcinoma. Originates from peritoneal cells. [medicaldb.blogspot.com]
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Hypothermia
We report a case of one-stage complete resection of a giant ICL with moderate hypothermia extracorporeal circulation and beating heart technique. The outcome of 36 months follow-up was very good. [ncbi.nlm.nih.gov]
Circulatory arrest with deep hypothermia avoids subjecting the liver and kidneys to periods of warm ischemia. [revespcardiol.org]
Tumors in the heart and inferior vena cava were successfully removed under deep hypothermia and circulatory arrest (Figures 6 and 7 ). The editor of Images in Cardiovascular Medicine is Hugh A. [circ.ahajournals.org]
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Soft Tissue Mass
Abdominal ultrasonography identified uterine leiomyoma and soft tissue masses. An abdominal CT demonstrated a continuous mass extending from the right internal and external iliac vein into the common iliac vein and inferior vena cava. [ncbi.nlm.nih.gov]
After neutralization of heparine, the unusual red soft tissue mass in the pelvis was resected with the left ovary and uterus. Massive hemorrhage (more than 8,000 mL) occurred during the resection. [atm.amegroups.com]
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Congestive Heart Failure
heart failure and sudden death. [ncbi.nlm.nih.gov]
At this time, we were unable to determine the possibility of high-output congestive heart failure caused by the pelvic AVF. [atm.amegroups.com]
The most common presenting features included congestive heart failure, features of venous obstruction and abdominal distention. [healio.com]
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Fishing
No cases showed loss of a hybridization signal by interphase FISH for the frequently deleted region of 7q22 in uterine leiomyomata. [ncbi.nlm.nih.gov]
Gastrointestinal
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Overeating
Upon laparoscopy multiple nodules were identified at the previous myomectomy scar, pelvic peritoneum, ovarian surface, and over the small bowel. [ncbi.nlm.nih.gov]
Although NRF2 activation is generally regarded as beneficial to human health, recent studies have identified that sustained NRF2 activation is over-represented in many cancers. [biochemsoctrans.org]
The Fourth Edition contains 3,494 full-color photographs, of which over 1,100 are new. This edition has three distinguished new editors—Joel K. Greenson, MD, Victor E. Reuter, MD, and Mark H. Stoler, MD—and many new contributors. [books.google.de]
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Abdominal Pain
Non-specific symptoms such as abdominal pain, vaginal bleeding, abdominal mass or gastrointestinal signs are described. Rare cases of malignant transformation have been reported. [ncbi.nlm.nih.gov]
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Chronic Abdominal Pain
We report the case of a 39-year-old woman with chronic abdominal pain and heavy dysmenorrhea due to endometriosis associated with LPD. She underwent an abdominal hysterectomy with bilateral salpingo-oophorectomy and omentectomy. [ncbi.nlm.nih.gov]
Cardiovascular
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Tachycardia
Intra-cardiac extension may represent a diagnostic challenge as it is usually misdiagnosed as a right atrial myxoma and may cause multiple symptoms, such as shortness of breath, tachycardia, chest pain, syncope, and even death. [ncbi.nlm.nih.gov]
The electrocardiogram showed sinus tachycardia and the chest radiography was normal. Arterial blood gases, serum troponin levels, and hematologic and coagulation parameters were within the normal range. [elsevier.pt]
After that, phenoxybenzamine might be chosen; it is an irreversible nonselective alpha blocker that can cause postural hypotension and reflex tachycardia, as well as gastrointestinal side effects. [dermatologyadvisor.com]
Workup
Imaging workup including trans-esophageal echocardiogram, cardiac catheterization, contrast-enhanced abdomen and pelvic CT scans, and cardiac MRI was performed for evaluation. [ncbi.nlm.nih.gov]
Clinical Testing and Workup A skin biopsy, which is the surgical removal and microscopic study of a small sample of skin tissue, is necessary in order to confirm the presence of leiomyomas. [rarediseases.org]
Treatment
Prompt surgical treatment with radical excision of pelvic and intravenous leiomyomatosis guarantees favorable outcomes and excellent prognosis with low rates of recurrence, whereas delayed diagnosis and treatment exposes to increased risk of congestive [ncbi.nlm.nih.gov]
Prognosis
KEYWORDS: Histogenesis; Immunohistochemistry; Prognosis; Uterus; intravenous leiomyomatosis (IVL) [ncbi.nlm.nih.gov]
Etiology
It is a challenge to clinicians when presenting a miliary pattern in preoperative radiologic imaging because it could be any other interstitial disease or infectious in etiology such as miliary tuberculosis. [ncbi.nlm.nih.gov]
The etiology of these neoplasms is not completely understood. Hysterectomy is the treatment of choice regardless the age of the patient. Uterine artery embolization is nowadays an alternative therapy. [manalclassroom.com]
Differential diagnosis at the time included both malignant and benign etiologies, such as leiomyosarcoma and IV leiomyomatosis. A PET/CT was done to assess malignant potential. [healio.com]
Epidemiology
Summary Epidemiology The prevalence is unknown. Over 200 families with HLRCC have been reported. Clinical description Disease onset can occur at any age, but is more common in young adults and elderly patients. [orpha.net]
Pathophysiology
[…] fumarate hydratase gene, conferring an increased risk of uterine and cutaneous leiomyomata as well as renal cancer The associated renal cell carcinoma is termed HLRCC associated renal cancer Sites Kidney Leiomyomas in uterus and skin in HLRCC syndrome Pathophysiology [pathologyoutlines.com]
PubMed/NCBI 4 Al-Talib A and Tulandi T: Pathophysiology and possible iatrogenic cause of leiomyomatosis peritonealis disseminata. Gynecol Obstet Invest. 69:239–244. 2010. [spandidos-publications.com]
Chinese Medical Journal. 2010; 123(22): 3367-3371 [Pubmed] 4 Pathophysiology and possible iatrogenic cause of leiomyomatosis peritonealis disseminata Al-Talib, A., Tulandi, T. [ijri.org]
Pathophysiology The FH gene mutation causes a decrease in the enzymatic activity of the enzyme. This important enzyme catalyzes the conversion of fumarate to L-malate. [dermatologyadvisor.com]
Pathology and pathophysiology of uterine smooth-muscle tumors. Environ Health Perspect. 2000;108(Suppl 5):779–784. 22. Toledo G, Oliva E. Smooth muscle tumors of the uterus: a practical approach. Arch Pathol Lab Med. 2008;132:595–605. 23. [jcadonline.com]
Prevention
The efficacy of using Gonadotropin releasing hormone agonists to prevent growth or recurrence of tumors in unresected or incompletely resected intravenous leiomyomatosis foci. [ncbi.nlm.nih.gov]
Although the syndrome may manifest life-threatening complications, outcomes may be improved by preventative family screening and surveillance, compelling early diagnosis. [casereports.bmj.com]
Treatment is focussed around the detection and prevention of cancer. Current advice is to have regular imaging of the kidneys. [dermnetnz.org]
Current medical research has not established a way of preventing Diffuse Leiomyomatosis of Uterine Corpus. [dovemed.com]
However, the suture attached to the tumor was held out of the atrium to prevent the tumor from going into the pulmonary artery as an embolus. [atm.amegroups.com]