scholarly journals Renal Epithelioid Angiomyolipoma in Children

2021 ◽  
Vol 8 (2) ◽  
pp. 20-26
Author(s):  
Dhruv Mahajan ◽  
Vishesh Jain ◽  
Sandeep Agarwala ◽  
Manisha Jana ◽  
Prashant P Ramteke
Keyword(s):  
Tuberous Sclerosis ◽  
Poor Prognosis ◽  
Tumor Metastasis ◽  
Renal Mass ◽  
Renal Tumor ◽  
Case Reports ◽  
Renal Angiomyolipoma ◽  
Epithelioid Angiomyolipoma ◽  
Left Flank

Renal angiomyolipoma is a rare cause of renal tumor in children. Most are associated with tuberous sclerosis, and the classic type is observed more commonly. Epithelioid angiomyolipoma is even rarer with only limited case reports and series published in literature, most of which are of adult patients. We describe a 12-year-old boy, a diagnosed patient of tuberous sclerosis, who presented with pain in the left flank. On evaluation, it was found to have a left renal mass with the clinical picture suggestive of renal cell carcinoma. Partial nephrectomy was performed and histopathology revealed epithelioid angiomyolipoma. The child was asymptomatic at follow-up after 3 months. Only a few such cases in children are found in literature, which are discussed alongside. Differential diagnosis of this rare tumor must be kept in mind in a renal tumor as surgery is generally curative in this possibly malignant tumor. Metastasis confers a poor prognosis. Chemotherapy is generally not effective, although various regimens have been tried. Tumor recurrence must be kept in mind and a follow-up after apparent complete remission is of paramount importance.

Aspiration Biopsy in Renal Angiomyolipoma

1989 ◽  
Vol 30 (4) ◽  
pp. 381-382 ◽  
Author(s):  
M. Taavitsainen ◽  
L. Krogerus ◽  
S. Rannikko
Keyword(s):  
Computed Tomography ◽  
Renal Mass ◽  
Aspiration Biopsy ◽  
Renal Angiomyolipoma ◽  
Ultrasound Guided ◽  
Open Biopsy ◽  
Safe Method ◽  
Imaging Results ◽  
Mass Lesions

Ultrasound guided aspiration biopsy of renal mass lesions was performed in 56 patients in whom renal cell carcinoma could not be excluded due to equivocal imaging results. Cytology showed non-malignant mesenchymal cells consistent with angiomyolipoma in 12 patients. In 5 of these patients the diagnosis was ensured by open biopsy. No nephrectomy was performed. In 6 of the 7 patients not operated upon the tumours remained unchanged with no evidence of malignancy during a 3–36 months' follow-up period. Aspiration biopsy is an easy and safe method for diagnosing renal angiomyolipoma in cases where computed tomography is not diagnostic for this disease.

scholarly journals Nephron Sparing Surgery for Renal Angiomyolipoma with Inferior Vena Cava Thrombus in Tuberous Sclerosis

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Adrien Riviere ◽  
Thomas Bessede ◽  
Jean-Jacques Patard
Keyword(s):  
Inferior Vena Cava ◽  
Tuberous Sclerosis ◽  
Inferior Vena ◽  
Vena Cava ◽  
Nephron Sparing Surgery ◽  
Renal Angiomyolipoma ◽  
Nephron Sparing ◽  
Mean Size ◽  
The Mean

Introduction. Angiomyolipoma is a common benign renal tumor. It is associated with Tuberous Sclerosis Complex (TSC) in 20% of patients. Angiomyolipomas are classically multiple, bilateral, and growing; they may lead to complications such as Wunderlich syndrome or, in rare cases, to venous extension.Observation. a 74-year-old woman with TSC presented with an angiomyolipoma of the right kidney with inferior vena cava (IVC) fatty thrombus. She underwent partial nephrectomy and thrombectomy. After a 7-year follow-up there was no evidence of recurrence or metastasis and her renal function was preserved.Review of Literature. It is the 44th reported angiomyolipoma associated with IVC thrombus. The mean size of angiomyolipomas was 86.1 mm and 67.4% of patients were symptomatic. Pulmonary embolism was found in 6 patients. There were 2 cases of recurrence/metastatic outcome after radical nephrectomy and thrombectomy. They were associated with epithelioid form. The mean size of epithelioid tumors was significantly bigger than in classical angiomyolipomas (127.1 mm versus 82.6 mm,P=0.037). With a median follow-up of 12 months, 91.3% of patients were recurrence and metastasis free, with 3 cases of nephron sparing surgery.Conclusion. Nephron sparing surgery for angiomyolipoma with IVC fatty thrombus can be safely performed in TSC, even in sporadic angiomyolipoma.

scholarly journals Spontaneous rupture of enormous renal angiomyolipoma in a pregnant tuberous sclerosis patient: a rare case

2020 ◽  
Vol 21 (1) ◽  
Author(s):  
Zechuan Liu ◽  
Yinghua Zou ◽  
Tianshi Lv ◽  
Haitao Guan ◽  
Zeyang Fan
Keyword(s):  
Tuberous Sclerosis ◽  
Rare Complication ◽  
Arterial Embolization ◽  
Renal Angiomyolipoma ◽  
Case Presentation ◽  
Selective Arterial Embolization ◽  
Medical Termination ◽  
The Status ◽  
First Time

Abstract Background Renal angiomyolipoma (RAML) is a rare benign kidney tumour comprised of adipose tissue, smooth muscle, and blood vessels. It can cause fatal complications if it ruptures. Although there have been reports of RAMLs rupturing, it is unusual to see RAMLs rupture during pregnancy, especially in pregnant women with tuberous sclerosis (TSC). Moreover, we reported a rare complication after selective arterial embolization (SAE) for the first time, which called aseptic liquefaction necrosis. Case presentation The case is a 16-week-pregnant woman with TSC who presented with severe flank pain, which was secondary to the rupture of a large, previously unknown RAML. This was confirmed by emergency computed tomography and successfully treated with selective arterial embolization after the patient received counselling and provided prior informed written consent for medical termination of pregnancy (MTP). The patient underwent abortion 3 weeks after the SAE. The patient required drainage 2 months after the SAE because of aseptic liquefaction necrosis. During follow-up, the patient’s lesion remained stable. Conclusion RAML rupture is a rare but rather serious complication in pregnant tuberous sclerosis patients. Selective arterial embolization (SAE) should be performed immediately, and the status of the pregnancy needs to be assessed by a multidisciplinary team. We also report for the first time the rare complication of aseptic liquefaction necrosis after SAE of RAML, for which percutaneous drainage is effective.

scholarly journals A Bladder Wall Angiomyolipoma as a Manifestation of Tuberous Sclerosis: First Case Report

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Mehmet Kalkan ◽  
Coşkun Şahin ◽  
Ömer Etlik ◽  
Ergun Uçmaklı
Keyword(s):  
Tuberous Sclerosis ◽  
Left Kidney ◽  
Bladder Wall ◽  
Hypovolemic Shock ◽  
Renal Angiomyolipoma ◽  
Lower Pole ◽  
Immunohistochemical Examination ◽  
First Case ◽  
Wall Mass

A 21-year-old female patient admitted to the emergency department complaining of left side pain. Hypovolemic shock, which was probably caused by retroperitoneal bleeding from left sided renal angiomyolipoma, was developed. Abdominal computed tomography showed multiple fat containing lesions in different, regions including right bladder wall, lower pole of left kidney, and right kidney. Some lesions compatible with tuberous sclerosis such as angiofibromas, Shagreen patches, myocardial, and brain hamartomas were also detected. Bladder wall mass showing intra- and extravesical extensions was seen at exploration. We removed the tumor completely preserving bladder trigone. Angiomyolipoma located at lower pole of left kidney was also removed. Diagnosis of bladder angiomyolipoma was confirmed by the immunohistochemical examination. Recurrent or residual mass was not detected at the three-months-follow-up. We report the first case of bladder angiomyolipoma confirmed by histopathologically as a tuberous sclerosis.

Metastatic renal epithelioid angiomyolipoma arising after a 23-year follow-up of sporadic renal angiomyolipoma

2013 ◽  
Vol 3 (3) ◽  
pp. 169-172
Author(s):  
Yoshinori Ikehata ◽  
Toshiaki Tanaka ◽  
Megumi Hirobe ◽  
Jiro Hashimoto ◽  
Hiroshi Kitamura ◽  
...  
Keyword(s):  
Renal Angiomyolipoma ◽  
Epithelioid Angiomyolipoma

scholarly journals A Scary Onset of a Rare and Aggressive Type of Primary Breast Sarcoma: A Case Report

2016 ◽  
Vol 9 (3) ◽  
pp. 796-801 ◽  
Author(s):  
Inês Ramalho ◽  
Sara Campos ◽  
Teresa Rebelo ◽  
Margarida Figueiredo Dias
Keyword(s):  
Poor Prognosis ◽  
Case Reports ◽  
Relevant Literature ◽  
Phyllodes Tumor ◽  
Left Breast ◽  
Breast Sarcoma ◽  
Systemic Dissemination ◽  
High Grade Sarcoma ◽  
Histological Characteristics

Primary breast sarcoma, arising from connective tissue within the breast, is extremely rare, accounting for less than 1% of all primary breast malignancies and no more than 5% of all sarcomas. The rarity of this pathology limits most studies to case reports and small retrospective studies, which has led to a lack of consensus on the clinical management. We report a clinical case of a 52-year-old woman, perimenopausal, previously healthy, with regular breast surveillance, who presented with a large (>20 cm) and rapidly expanding hypervascularized tumor of the left breast developed over 10 days, with a very thin preulcerative skin over the last 4 days. There was no systemic dissemination. The patient was submitted to total mastectomy and excision of axillary adenopathy. The tumor was diagnosed histologically as malignant phyllodes tumor associated with areas of high-grade sarcoma. Due to rapid growth and aggressive histological characteristics, adjuvant chemotherapy and radiotherapy were performed. There is a lot of evidence that tumors larger than 5 cm are associated with a poor prognosis. Despite the poor prognosis associated with this aggressive entity, the patient had no recurrence during 5 years of follow-up. We review the relevant literature about primary breast sarcomas.

scholarly journals Postembolization Intratumoral Chronic Bleeding, without the Classic CT Feature of Active Extravasation, in Tuberous Sclerosis Complex-Related Renal Angiomyolipoma: Two Case Reports

2018 ◽  
Vol 8 (2) ◽  
pp. 112-119
Author(s):  
Xixi Zhang ◽  
Ryohei Kuwatsuru ◽  
Hiroshi Toei ◽  
Daisuke Yashiro ◽  
Shingo Okada ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Case Reports ◽  
Renal Angiomyolipoma ◽  
Contrast Extravasation ◽  
Active Extravasation ◽  
Enhanced Computed Tomography ◽  
Intratumoral Bleeding ◽  
Abdominal Fullness ◽  
Chronic Hematoma

Two patients with tuberous sclerosis complex each had multiple bilateral renal angiomyolipomas. After undergoing embolization for a ruptured angiomyolipoma, patient 1 experienced long-lasting abdominal fullness; contrast-enhanced computed tomography (CECT) revealed a large chronic hematoma without contrast extravasation. Patient 2 underwent embolization for the largest right renal angiomyolipoma which contained a chronic hematoma. 2 weeks later, the symptom of abdominal fullness presented, and CECT revealed that the preexisting hematoma had enlarged without contrast extravasation. In both cases, a second embolization of the angiomyolipomas resulted in shrinking of the intratumoral hematomas and alleviation of the associated symptoms. Therefore, chronic postembolization intratumoral bleeding from renal angiomyolipoma may present as a persistently large or growing hematoma with an associated symptom of abdominal fullness but without the typical CECT feature of active extravasation.

Continuous low-dose everolimus shrinkage tuberous sclerosis complex-associated renal angiomyolipoma: a 48-month follow-up study

2018 ◽  
Vol 67 (3) ◽  
pp. 686-690 ◽  
Author(s):  
Chang-Ching Wei ◽  
Jeng-Daw Tsai ◽  
Ji-Nan Sheu ◽  
Sung-Lang Chen ◽  
Teng-Fu Tsao ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Low Dose ◽  
Benign Neoplasm ◽  
Volume Reduction ◽  
Response To Treatment ◽  
Renal Angiomyolipoma ◽  
Group I ◽  
Group Ii

Tuberous sclerosis complex (TSC) is a rare disease that causes multisystem benign neoplasm, induced by dysregulation of the mammalian target of the rapamycin pathway (mTOR). This study aimed to examine the effects of continuous low-dose everolimus, a potent and selective inhibitor of mTOR, on the treatment of TSC-associated renal angiomyolipoma (AML). Between July 2013 and August 2017, 11 patients with TSC-AML were enrolled for an everolimus therapy protocol. An oral everolimus dose starting at 2.5 mg daily was gradually increased to 5.0 mg daily. All patients were evaluated using MRI or CT scanning at baseline, 12, 24, 36 and 48 months after the start of treatment for measuring changes of renal AML mass volume. Everolimus therapy resulted in significant shrinkage of TSC-AML volume after 48 months follow-up. Serum levels of everolimus were subdivided into group I (<8 ng/mL, n=6) and group II (>8 ng/mL, n=5). The volume reduction rates were 10.6%–65.2% in group I and 42.5%–70.6% in group II. To evaluate the response to treatment, three of six (50%) were responders in group I, and all the patients in group II (5/5, 100%) were responders. The differences in AML volume reduction between the groups were statistically significant at 12 months (p=0.011), 24 months (p=0006), 36 months (p=0.014) and 48 months (p=0.05). These results suggest that continuous low-dose everolimus therapy (2.5–5 mg daily) might be effective in shrinking TSC-AML volume and minimizes adverse effects and subsequent reducing medical costs.

1139 Everolimus for renal angiomyolipoma associated with tuberous sclerosis complex (TSC): EXIST-2 3-year follow-up

2014 ◽  
Vol 13 (1) ◽  
pp. e1139 ◽  
Author(s):  
J.J. Bissler ◽  
J.C. Kingswood ◽  
E. Radzikowska ◽  
B.A. Zonnenberg ◽  
M. Frost ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Renal Angiomyolipoma

scholarly journals Renal Endometriosis Mimicking Cystic Renal Tumor: Case Report and Literature Review

2021 ◽  
Vol 8 ◽  
Author(s):  
Ye Yang ◽  
Xinxin Zhao ◽  
Ying Huang
Keyword(s):  
Renal Mass ◽  
Clinical Symptoms ◽  
Renal Tumor ◽  
Cystic Mass ◽  
Case Presentation ◽  
Contrast Enhanced ◽  
The Right ◽  
Enhanced Computed Tomography ◽  
Contrast Enhanced Computed Tomography

Background: Endometriosis mainly affects female pelvic tissues and organs, and the presence of endometriosis in the kidney is extremely rare.Case Presentation: We report a case of a 48-year-old woman who presented with intermittent hematuria. She was found to have a cystic mass on renal ultrasonography, and contrast-enhanced computed tomography (CT) showed slight enhancement of the cystic wall and septa. These findings were indicative of cystic renal tumor. The patient subsequently underwent partial right nephrectomy. Histopathology revealed endometriosis of the right renal parenchyma. The patient recovered well and had no evidence of a recurrent renal mass at the 3 months' follow up.Conclusion: The possibility of renal endometriosis should be considered in a female patient with a cystic renal mass and clinical symptoms related to the menstrual cycle.

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