Primitive neuroectodermal tumour of the kidney: An unusual case mimicking renal angiomyolipoma with minimal fat

Primitive neuroectodermal tumour (PNET) is a highly aggressive neoplasm that develops classically in the central nervous system. PNET of the kidney (rPNET) is extremely rare. Recently, a 23-yearold woman complained of left flank pain and intermittent hematuria for 3 months and was admitted to our hospital. A computed tomography (CT) scan and magnetic resonance imaging demonstrated a 5.1 × 4.4-cm heterogenous mass with unconspicuous reinforcement in the upper pole of the left kidney. F18-FDG positron emission tomography CT (PET-CT) revealed the mass as a benign lesion with internal extensive bleeding. Renal angiomyolipoma with minimal fat was diagnosed. Three months later, a CT scan showed that the mass shrank to 3.1 × 2.6 cm and nephron-sparing surgery of the left kidney was performed at the patient’s request. However, histologic features and immunohistochemical analysis confirmed the diagnosis of rPNET. Five cycles of combined chemotherapy were executed. At the 11-month follow-up, the patient showed no evidence of metastasis or recurrence.

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Primitive neuroectodermal tumour with synchronous ipsilateral clear cell carcinoma of the kidney

BMJ Case Reports ◽

10.1136/bcr-2018-224273 ◽

2018 ◽

Vol 11 (1) ◽

pp. e224273

Author(s):

Mohamed Taha ◽

Nur M Mohammed ◽

Stephen Crowther ◽

Rustom P Manecksha ◽

Arun Z Thomas

Keyword(s):

Cell Carcinoma ◽

Clear Cell ◽

Clear Cell Carcinoma ◽

Left Kidney ◽

Disease Recurrence ◽

Cell Renal Cell Carcinoma ◽

Primitive Neuroectodermal Tumour ◽

Neuroectodermal Tumour ◽

First Case ◽

Solid Lesions

We report the first case of a synchronous ipsilateral primitive neuroectodermal tumour (PNET) and clear cell renal cell carcinoma of the kidney. A 37-year-old man presented to the emergency department with a 24-hour history of colicky abdominal pain and visible haematuria. He had no relevant surgical or medical history. Physical examination was unremarkable apart from mild left flank tenderness. Triphasic CT of the abdomen and pelvis showed two solid lesions in the left kidney. Further staging CT of the chest showed no evidence of local or distal metastasis. He subsequently underwent laparoscopic radical nephrectomy. Pathological analysis of the kidney showed two synchronous renal tumours, a clear cell carcinoma and PNET of the kidney. The patient received adjuvant chemotherapy according to Ewing’s sarcoma chemotherapy protocol. Surveillance CT scans at 3, 6 and 12 months showed no evidence of disease recurrence or metastasis.

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Primitive neuroectodermal tumour of the central nervous system associated with malignant rhabdoid tumour of the kidney: report of a case

Histopathology ◽

10.1111/j.1365-2559.1986.tb02517.x ◽

1986 ◽

Vol 10 (6) ◽

pp. 643-650 ◽

Cited By ~ 20

Author(s):

A.J. HOWAT ◽

M.F. GONZALES ◽

K.D. WATERS ◽

P.E. CAMPBELL

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Rhabdoid Tumour ◽

Primitive Neuroectodermal Tumour ◽

Neuroectodermal Tumour ◽

The Central Nervous System

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An ovary in luteal phase mimicking common iliac lymph node metastasis from a primary cutaneous peripheral primitive neuroectodermal tumour as revealed by 18-fluoro-2-deoxyglucose positron emission tomography

British Journal of Radiology ◽

10.1259/bjr/95232584 ◽

2005 ◽

Vol 78 (928) ◽

pp. 343-345 ◽

Cited By ~ 11

Author(s):

K-C Ho ◽

K-K Ng ◽

T-C Yen ◽

H-H Chou

Keyword(s):

Positron Emission Tomography ◽

Lymph Node ◽

Lymph Node Metastasis ◽

Luteal Phase ◽

Emission Tomography ◽

Primitive Neuroectodermal Tumour ◽

Node Metastasis ◽

Iliac Lymph Node ◽

Neuroectodermal Tumour ◽

Positron Emission

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Primitive neuroectodermal tumour of the kidney presenting as diplopia secondary to skull metastasis

BMJ Case Reports ◽

10.1136/bcr-2019-230827 ◽

2019 ◽

Vol 12 (12) ◽

pp. e230827

Author(s):

Shanmugasundaram Rajaian ◽

Murugavaithianathan Pragatheeswarane ◽

Karrthik Krishnamurthy ◽

Srinivas Chakravarthy Narasimhachar

Keyword(s):

Excision Biopsy ◽

Team Approach ◽

Tissue Mass ◽

Primitive Neuroectodermal Tumour ◽

Skull Metastasis ◽

Inferior Surface ◽

Neuroectodermal Tumour ◽

Positron Emission ◽

The Right ◽

The Brain

A 20-year-old man presented to the department of neurology with diplopia, occipital headache and right flank pain for 1-week duration. CT of the brain revealed skull metastasis with heterogeneously enhancing dural-based mass lesion at the occipital region. Positron emission tomography revealed tracer avid soft tissue mass involving the upper pole of the right kidney with loss of fat planes with the inferior surface of the liver. Multidisciplinary team approach was discussed. He underwent palliative nephrectomy with lymph nodal mass excision. Biopsy from the renal mass was suggestive of primitive neuroectodermal tumour. He developed progressive liver metastases in spite of adjuvant chemotherapy denoting very aggressive disease.

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Primary Pleural Angiosarcoma in a 63-Year-Old Gentleman

Case Reports in Pulmonology ◽

10.1155/2013/974567 ◽

2013 ◽

Vol 2013 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Ahmed Abu-Zaid ◽

Shamayel Mohammed

Keyword(s):

Computed Tomography ◽

Ct Scan ◽

Histopathological Examination ◽

Case Reports ◽

Immunohistochemical Analysis ◽

Whole Body ◽

Cytologic Examination ◽

Ct Guided ◽

Positron Emission ◽

The Right

Primary pleural angiosarcomas are extremely rare. As of 2010, only around 50 case reports have been documented in the literature. Herein, we report the case of a 63-year-old gentleman who presented with a 3-month history of right-sided chest pain, dyspnea, and hemoptysis. Chest X-ray showed bilateral pleural effusion with partial bibasilar atelectasis. Ultrasound-guided thoracocentesis showed bloody and exudative pleural fluid. Cytologic examination was negative for malignant cells. An abdominal contrast-enhanced computed tomography (CT) scan showed two right diaphragmatic pleural masses. Whole-body positron emission tomography/computed tomography (PET/CT) scan showed two hypermetabolic fluorodeoxyglucose- (FDG-) avid lesions involving the right diaphragmatic pleura. CT-guided needle-core biopsy was performed and histopathological examination showed neoplastic cells growing mainly in sheets with focal areas suggestive of vascular spaces lined by cytologically malignant epithelioid cells. Immunohistochemical analysis showed strong positivity for vimentin, CD31, CD68, and Fli-1 markers. The overall pathological and immunohistochemical features supported the diagnosis of epithelioid angiosarcoma. The patient was scheduled for surgery in three weeks. Unfortunately, the patient died after one week after discharge secondary to pulseless ventricular tachycardia arrest followed by asystole. Moreover, we also present a brief literature review on pleural angiosarcoma.

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Hepatic epithelioid angiomyolipoma: a case report

International Surgery Journal ◽

10.18203/2349-2902.isj20210020 ◽

2021 ◽

Author(s):

Ana Logrado ◽

Raquel Pereira ◽

Júlio Constantino ◽

Milene Sá ◽

Jorge Pereira ◽

...

Keyword(s):

Left Kidney ◽

Immunohistochemical Analysis ◽

Tumor Board ◽

Pathology Report ◽

Renal Angiomyolipoma ◽

Epithelioid Angiomyolipoma ◽

Predominant Component ◽

Hepatic Nodule ◽

Rare Lesion ◽

Epithelioid Variant

Angiomyolipoma (AML) is a rare benign solid tumor, of mesenchymal origin. Angiomyolipomas can be subdivided, according to their predominant component. The predominance of epithelioid cells characterizes the epithelioid variant (EAML). These express a more aggressive clinical behaviour with a greater potential for malignant transformation. In the absence of specific radiological features, the diagnosis of hepatic EAML depends on the pathological and immunohistochemical study. We present the case of an 80 years old female with a computed tomography (CT) scan showing a hepatic nodule at the transition of segments 5 and 8, and a left kidney nodule previously biopsied and confirmed to be a renal angiomyolipoma. A liver nodule biopsy was performed, whose histology revealed a probable gastrointestinal stromal tumor (GIST). The patient underwent a 5/8 subsegmentectomy and pathology report revealed epithelioid angiomyolipoma. After discussion of the case in the multidisciplinary tumor board, it was decided to perform a left nephrectomy, whose pathology revealed leiomyomatous angiomyolipoma. No further treatment and surveillance on outpatient clinic were decided. Hepatic EAML is a rare lesion that, although mostly benign, can exhibit malignant behavior with distant metastasis and local invasion. Its identification is of paramount importance. The definitive diagnosis is only possible through histological and immunohistochemical analysis. Additional studies are needed in order to establish diagnostic criteria and predictive characteristics of malignancy.

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Angiomiolipoma renale: Nostra esperienza in 14 casi sottoposti a terapia chirurgica

Urologia Journal ◽

10.1177/039156039706401s29 ◽

1997 ◽

Vol 64 (1_suppl) ◽

pp. 117-122

Author(s):

G. Deiana ◽

D. Belussi ◽

R. Hurle ◽

A. Losa ◽

E. Micheli ◽

...

Keyword(s):

Ct Scan ◽

Tuberous Sclerosis ◽

Left Kidney ◽

Correct Diagnosis ◽

Diagnostic Error ◽

Renal Angiomyolipoma ◽

Retroperitoneal Hemorrhage ◽

Renal Masses ◽

Minimum Age ◽

Operative Ultrasound

Renal angiomyolipoma (AML) is an uncommon hamartomatous benign tumour and may present in an isolated form or associated with tuberous sclerosis (Bourneville's disease). Since there is no specific symptomatology when dimensions are small or medium, discovery is often accidental and the tumour may sometimes grow to a considerable size. In the symptomatic forms or larger tumours, surgery may be indicated due to the risk of retroperitoneal hemorrhage, both spontaneous and secondary to lumbar trauma, however slight. As the tumour is benign, kidney-sparing surgery should be as conservative as possible. For this purpose, ultrasound and CT scan are considered essential for a differential diagnosis between AML and parenchymal renal masses. In our experience with 14 cases (12 women and 2 men; minimum age 31 years, maximum 62 years) of monolateral renal AML (10 right kidney AML and 4 left kidney AML) in patients not suffering from tuberous sclerosis, treated surgically between August 1988 and February 1997, the pre-operative ultrasound and CT scan gave a correct diagnosis in 7 cases, a suggestion of renal carcinoma in 5 cases while leaving doubts in 2 cases. The two methods of investigation therefore proved not to be error-free. Following instrumental diagnosis, 12 patients underwent enucleoresection of the tumour while the remaining two underwent extensive nephrectomy. The presence of hemorrhagic areas with AML was considered to be the frequent cause of pre-operative diagnostic error.

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