Wilms’ tumor (nephroblastoma) is extremely rare in adults, skeletal metastasis being still rarer. The clinical course of adult Wilms’ tumor is very aggressive. The present case is a rare blastemal predominant adult Wilms’ tumor presenting with skeletal metastasis. We report a case of 19-year-old female presented with severe low backache and colicky left loin pain of 3 months and progressive weakness of 15 days duration. Magnetic resonance image (MRI) of lumbosacral spine was reported as spinal metastasis with right renal mass. The patient underwent right radical nephrectomy and the tumor was histopathologically confirmed as adult Wilms’ tumor. In case of adult Wilms’ tumor, distant metastasis may be the first presentation and this possibility should be considered when an adult patient presents with flank pain and a renal mass.
Adult Wilms’ tumor; renal tumor; skeletal metastasis
Wilms' tumor is the commonest primary malignant renal tumor in childhood. Rarely, it may present in the adult age group.
We report a 48-year-old male presenting with flank pain and haematuria. Abdominal ultrasound revealed a right renal mass measuring 11 × 10 cms, and a clinical diagnosis of renal cell carcinoma was made. Nephrectomy was performed, and a final diagnosis of adult Wilms' tumor was made based on the criteria proposed by Kilton et al.
The possibility of an adult Wilms' tumor should be considered when a patient presents with pain in the flank and a renal mass. Rarity of the tumor favors documentation in literature.
A study was undertaken to determine whether there are any features of retroperitoneal tumors in children that might be demonstrated on roentgenograms to aid in identifying them preoperatively. Study was limited to Wilms' tumor of the kidney and neuroblastoma.
Calcification was found in 57 per cent of the neuroblastomas and in only 12 per cent of Wilms' tumors. Calcifications in neuroblastomas differed from those in Wilms' tumors. Calcification in neuroblastoma was more frequent in older children than in the younger ones.
The kidney was frequently displaced by both types of tumor. However, the neuroblastoma always displaced the kidney downward, or downward and slightly outward.
In most instances, the Wilms' tumor also displaced the kidney downward and outward, but in some instances upward and medially. This, of course, depended upon the site of origin of the tumor.
There was a distortion of the intrarenal structures in 75 per cent of the cases of neuroblastoma and in 71 per cent of the cases of Wilms' tumor.
Wilms' tumor is one of the most frequent malignant neoplasms in childhood. Advances in treatment modalities such as the combination of chemoradiation therapy with surgery have enhanced overall survival. However, recurrence of Wilms' tumor is still a problem. In this case, a 28-year-old female had experienced intermittent abdominal pain, and the computed tomography scan showed a huge pelvic mass. The patient had a history of radical nephrectomy for Wilms' tumor with concurrent chemotherapy at the age of three. The pelvic mass was resected in February 2010 and was confirmed to be a recurrent Wilms' tumor. The recurrent tumor showed a classic triphasic Wilms' tumor growth pattern with frequent mitoses and tumor necrosis. Our case is an extraordinary case of a long-delayed recurrent Wilms' tumor after 25 years, which is the longest disease-free interval ever reported. The possible effects of chemotherapy as well as some other mechanisms of this late relapse are discussed.
Drug therapy; Late recurrence; Therapeutics; Wilms tumor
Primary renal Ewing sarcoma/PNET is an uncommon and very aggressive tumor.
Presentation of case
: We report the case of a young woman who underwent nephrectomy for a renal mass from unknown etiology. Histologic analysis found small tumoral cells in rosette formation, and immunohistochemical staining was positive for CD99, and focally positive for vimentin and Protein S-100. A post operative abdominal computed tomography (CT) scan revealed a residual renal processus with hepatic wounds and abdominal metastatic nodes.
After 6 cycles of chemotherapy including Vincristine, Doxorubicin, Cyclophosphamide, the response was considered as good.
Ewing's sarcoma/PNET of kidney is a member of the family of small round cell tumors and it should be differentiated from Wilms tumor, neuroblastoma, rhabdomyosarcoma and lymphoblastic lymphoma. The principle management of its treatment have been extrapolated from the treatment of osseous Ewing sarcoma of bone.
Despite aggressive treatment, primary renal Ewing sarcoma/PNET has a poor prognosis. It requires a multidisciplinary approach including oncologists, urologists and radiation oncologists.
PNET of kidney; Complete response
We report a case of renal hydatid cyst in a 25-year-old male who presented with hydatiduria. Intravenous pyelography revealed presence of a space-occupying lesion in the lower pole of right kidney with curvilinear calcifications. Ultrasound, computed tomography and MRI were suggestive of hydatid cyst in the right kidney. Patient underwent right-sided nephrectomy. Passage of hydatid cysts in urine is an exceedingly rare occurrence. Urinary tract involvement develops in 2–4% of all cases of hydatid cyst. Hydatiduria is an extremely rare manifestation of renal hydatid cyst. We report such a case with emphasis on IVU, sonographic, CT and MRI findings.
Hydatid cyst; hydatiduria; hydronephrosis; multiloculated
Adrenocortical carcinoma (ACC) is a rare malignancy, especially in children. The overall incidence is approximately 2 cases per million per year. In children, the incidence is 0.3 cases per million per year, except in southern Brazil where the incidence is 3.4–4.2 cases per million per year. We describe a giant nonfunctioning metastasized ACC in a 6-year-old girl who presented with a history of increasing abdominal girth incidentally noticed by her mother since 1 week. Ultrasound abdomen showed a large right suprarenal tumor with calcifications and necrosis. Empty left renal fossa and compensatory enlarged right kidney were seen. Computed tomography (CT) scan revealed a large heterogenously enhancing right suprarenal mass with calcification and necrosis with pulmonary metastasis. Histopathology report from the right suprarenal mass revealed an ACC. With a stage IV disease, the patient died after 2 months from diagnosis.
Adrenocortical carcinoma; adrenocortical tumor; nonfunctioning
Sarcomatoid renal cell carcinomas of the kidney are rare neoplasms constituting about 1-5% of all renal malignant neoplasms. These are aggressive tumors and are commonly associated with conventional (clear cell) renal cell carcinomas, but cases associated with chromophobe renal cell carcinomas are sparse. Cytological features of such lesions have rarely been reported. Here, we report a unique case of a 48-year-old male patient who presented with right flank lump and pain. A fine needle aspiration was performed from the lesion under ultrasound guidance and a cytological diagnosis of pleomorphic sarcoma was made. A right-sided radical nephrectomy was carried out and subsequent histopathology revealed a sarcomatoid renal cell carcinoma with wide areas of necrosis coexisting with chromophobe renal cell carcinoma with calcification. Differentiation of pleomorphic sarcoma from a sarcomatoid renal cell carcinoma is, thus, challenging from cytopathology smears and the differential diagnoses should always be borne in mind while giving a cytopathological opinion.
Cytology; kidney; pleomorphic sarcoma; sarcomatoid chromophobe renal cell carcinoma
Renal primitive neuroectodermal tumor (rPNET) as a member of Ewing’s sarcoma family is extremely rare and usually occurs in children and young adults. Most literature about rPNET was isolated case reports.
We reported a case of 45-year-old man with the complaint of right flank pain. Computerized tomography (CT) scan demonstrated a large substantive tumor involving the lower pole of the right kidney. Then the patient underwent radical nephrectomy. Pathologic characteristics and immunohistochemical analysis confirmed the diagnosis of rPNET. Additionally, the patient received three cycles of chemotherapy, and was still alive without metastasis at 15-months follow-up.
rPNET is rare and presents aggressive clinical behavior and worse prognosis. We expect that further awareness and study of this rare tumor can be had by presenting our case.
Primitive neuroectodermal tumor; Kidney carcinoma; Ewing’s sarcoma
Wilms’ tumor is rare in adults. Though the approach to diagnosis and treatment of adult Wilms’ tumor (AWT) is closely modeled on recommendations for childhood Wilms’ tumor, views differ on how aggressive the treatment should be. We report a case of a 37-year-old with Stage III favorable histology AWT. A radical nephrectomy was performed and the patient was due for chemotherapy. Recent advances, controversies and current recommendations in the treatment of AWT are discussed.
Adult; Wilms’ tumor; Kidney
Teratoid Wilms’ tumor is an extremely rare renal tumor. We report a case of unilateral teratoid Wilms’ tumor in a 4-year-old girl. The patient was admitted with a right-sided abdominal mass. The mass was arising from the right kidney. Radical nephrectomy was done and the patient had an uneventful recovery. Histopathology report showed teratoid Wilms’ tumor.
Chemotherapy; radical nephrectomy; teratoid Wilms’ tumor; teratoma; unilateral; Wilms’ tumor
Mucinous cystic neoplasms (MCNs) make up a morphologic family of similar appearing tumors arising in the ovary and various extraovarian organs such as pancreas, hepatobiliary tract and mesentery. MCNs of the pancreas occur almost exclusively in women. Here, we report a rare case of MCN in a male patient. A 39-year-old man was admitted to our hospital with the chief complaint of back pain. Abdominal computed tomography revealed a multilocular cyctic mass 6.3 cm in diameter in the pancreatic tail. In addition, the outer wall and septae with calcification were demonstrated in the cystic lesion. On magnetic resonance imaging , the cystic fluid had low intensity on T1-weighted imaging and high intensity on T2-weighted imaging. Endoscopic retrograde cholangio-pancreatography (ERCP) showed neither communication between the cystic lesion and the main pancreatic duct nor encasement of the main pancreatic duct. Endoscopic ultrasonography revealed neither solid component nor thickness of the septae in the cystic lesion. Consequently, we performed distal pancreatectomy with splenectomy under the diagnosis of cystic neoplasia of the pancreas. Histopathologically, the cystic lesion showed two distinct component: an inner epithelial layer and an outer densely cellular ovarian-type stromal layer. Based on these findings, the cystic lesion was diagnosed as MCN.
Mucinous cystic neoplasm; male; pancreas.
Xanthogranulomatous pyelonephritis (XGP) is a rare event in children without any predisposing factor like calculi, obstruction or vesicoureteral reflux. In this case we report a four-year-old girl who presented with a renal mass, hematuria, flank pain, anemia and thrombocytopenia—these signs and symptoms misled us to Wilms tumor. Thrombocytopenia which is a strange event in XGP resolved after nephrectomy. Normal contra lateral kidney was infected four months after right nephrectomy. This suggests that these patients should be under strict surveillance and antibiotic prophylaxis as they are a high-risk group for urinary tract infection, and thrombocytopenia should be considered as a laboratory test finding in XGP.
Renal mass; thrombocytopenia; xanthogranulomatous pyelonephritis
The case was a 40-year-old female. She visited a local doctor with a chief complaint of right side abdominal pain. A right kidney tumor measuring 10 cm in diameter was observed in an abdominal Computed Tomography (CT) scan. Based on the CT image, the possibility of angiomiolipoma (AML) could not be ruled out, but a high maximum standardized uptake value (SUVmax) of 7.8 was observed in a Positron Emission Tomography CT (PET-CT) scan and there was a possibility of malignancy. We therefore performed a transperitoneal right radial nephrectomy. Although adhesion of the tumor to the duodenum and the inferior vena cava was observed, it was possible to perform an excision. The tumor accounted for a large proportion of the excised kidney; the surrounding areas had taken on a cyst-like structure, and the interior comprised grayish brittle tissue exhibiting solid growth. Histologically, gland-like and cyst-like structures composed of cylindrical cuboidal cells and mainly characterized by the solid growth of short fusiform-shaped and oval-shaped basophilic cells were observed, and we believed it was a synovial sarcoma. There were no malignant findings in the adrenal gland. There have been approximately 30 reported cases around the world of synovial sarcoma that developed in the kidney, and we herein report this case with bibliographic considerations.
Synovial sarcoma; Renal tumor; Renal mass; PET-CT; SYT-SSX
Metastatic myocardial calcification is a frequent cause of heart failure in hemodialysis patients. However, early detection is difficult, often resulting in a poor prognosis. A 47-year-old man with hemodialysis-dependent end-stage renal disease presented with progressive dyspnea. Levels of serum phosphate, calcium, and intact parathyroid hormone were poorly controlled. He developed pain in his right thumb 1 year before presentation, and the pain gradually increased and extended to the entire right hand. Hand radiography 1 month earlier had revealed significant progressive calcification. Echocardiography showed severe, diffuse hypokinesis and pericardial effusion as well as possible anterior myocardial calcification with high echogenicity. Chest computed tomography revealed a severely dilated heart with anterior massive myocardial calcification and a large amount of pericardial effusion, which was not detected on computed tomography performed 20 months earlier. The patient was diagnosed with heart failure associated with metastatic myocardial calcification and died suddenly 2 weeks later. This experience suggests that progressive metastatic calcification of the skin and subcutaneous tissue is useful for predicting myocardial calcification.
Cardiovascular disease; Hemodialysis/continuous renal replacement therapy; Mineral bone metabolism; Metastatic calcification; Heart failure; Hyperparathyroidism; Myocardium; Hand; Computed tomography
We present a rare case of a gastrointestinal stromal tumor (GIST) in the stomach with prominent calcification at presentation. A 61-year-old woman visited our hospital because of epigastric discomfort. A spherical calcified lesion with a diameter of about 30 mm was incidentally shown in the left upper quadrant on an abdominal X-ray. Computed tomography demonstrated that the tumor was growing from the upper gastric body, with calcification in the peripheral ring area. A laparoscopic partial gastrectomy was performed, and the resected specimen revealed a well-circumscribed tumor with exophytic growth from the gastric muscularis propria. Microscopic examination revealed spindle-shaped tumor cells with calcification and hemorrhage. Additionally, positive immunoreactivity of the tumor to KIT and CD34 and a low mitotic index resulted in the diagnosis of very low risk GIST. There are a few case reports of heavily calcified GIST, although solitary or punctate calcification of primary GIST has been reported in several case series. Dystrophic calcification of necrotic or degenerative tissue is the supposed cause of primary calcified GISTs. In contrast, appearance of calcification after administration of imatinib mesylate, which may be one indicator of disease response, is possibly caused by a different mechanism.
Gastrointestinal stromal tumor; Calcification; Stomach; Computed tomography; Imatinib mesylate
We report a case of tuberous sclerosis associated with two histologically different renal cell carcinomas (RCCs) and multiple angiomyolipomas (AMLs) in the same kidney. A 43-year-old female was admitted to our hospital with left flank pain and a huge palpable mass in the left flank area. Abdominal computed tomography revealed two concurrent RCCs and multiple AMLs in the left kidney. Because of the clinical suspicion of RCC, the patient underwent left radical nephrectomy. On gross examination, the total size of the resected left kidney was 30.5×17×8 cm. Microscopically, the upper pole tumor features were consistent with chromophobe RCC and the midpole tumor was a clear-cell RCC. The multifocal masses in the remaining remnant parenchyma were AMLs. Six months after surgery, the patient is healthy without signs of tumor recurrence.
Angiomyolipoma; Clear cell renal carcinoma; Renal cell carcinoma; Tuberous sclerosis
Extraskeletal osteosarcoma is a malignant mesenchymal soft tissue tumor without attachment to the bone, and is able to produce osteoid or cartilaginous matrix. This entity accounts for 1% of all soft tissue sarcomas. Thus far, less than 35 cases of bladder osteosarcomas have been reported in the literature. These tumors are associated with very poor prognosis. We report a case of primary osteosarcoma of the urinary bladder presenting with intermittent hematuria, dysuria, and right flank pain. Contrast-enhanced computed tomography scan of the abdomen, pelvis, and chest revealed a bladder mass and pulmonary metastases with specks of calcification.
Extraskeletal osteosarcoma; urinary bladder primary osteosarcoma; mesenchymal tumors
The simultaneous occurrence of renal cell carcinoma(RCC) and transitional cell carcinoma(TCC) in the same kidney is unusual. We report a 53-year-old man with ipsilateral synchronous renal adenocarcinoma and renal pelvic transitional cell carcinoma with severe hypercalcemia and a huge staghorn calculus in the opposite kidney. The patient was admitted to the hospital because of left flank pain and intermittent fever which he had had for 2 months. Computerized tomography revealed a huge stone in the right kidney and a mass in the upper pole with an irregular calcified pelvis in the left enlarged kidney. Left radical nephrectomy was done. A section of the specimen revealed a renal cell carcinoma located at the upper pole and a papillary transitional cell carcinoma arising from the renal pelvis. This is a rare case of combined renal malignancies.
Double chambered right ventricle is a rare congenital cardiac anomaly in which the right ventricle is divided into two chambers by an anomalous muscle bundle. The diagnosis of this disorder is difficult in adults. Calcification of the tricuspid valve is extremely rare, and very few cases have been reported. Most cases of tricuspid valve calcification had a congenital disorder with high pressure in the right ventricle.
We report a rare case of a 71-year-old Japanese woman who presented with chest discomfort, and was found to have a double chambered right ventricle with severe calcification of the tricuspid valve. This abnormality was found by echocardiography, and the diagnosis was confirmed by multislice cardiac computerized tomography, cardiac magnetic resonance imaging, and cardiac catheterization. Our patient rejected surgical repair, and medical therapy with carvedilol was effective to reduce her symptoms.
Calcification of the tricuspid valve is extremely rare, and considered to be due to high pressure in the right ventricle. To the best of our knowledge, there are no other reported cases of this combination of double chambered right ventricle and calcification of the tricuspid valve.
When performing a radiological assessment for a trauma case with associated head injury, a fragment of dense tissue detected near the craniovertebral junction would rapidly be assessed as a fractured bone fragment. However, if further imaging and evaluation of the cervical spine with computerised tomography (CT) did not demonstrate an obvious fracture, then the possibility of ligament calcification would be considered. We present a case involving a previously healthy 44-yearold man who was admitted following a severe head injury from a road traffic accident. CT scans of the head showed multiple intracranial haemorrhages, while scans of the cervical spine revealed a small, well-defined, ovoid calcification in the right alar ligament. This was initially thought to be a fracture fragment. Although such calcification is uncommon, accident and emergency physicians and radiologists may find this useful as a differential diagnosis in patients presenting with neck pain or traumatic head injury.
cervical spine; calcification; computerised tomography; injury; medical sciences
There are few case reports of thymoma with a thymic cyst. Such an association renders it difficult for any pathologist to differentiate from other neoplasms, such as a cystic thymoma.
A 50-year-old Berber woman from Morocco was admitted with a chronic cough of more than 10 years duration. Her medical history and physical examination were normal. Anterior chest radiography demonstrated a calcified opacity in her right anterior mediastinum. A chest-computed tomogram revealed a round cystic tumor, with significant calcification in her right anterior mediastinum. A surgical exploration was performed. The tumor seemed to be a well-encapsulated and totally calcified lesion, arising from the right lobe of her thymus. It was removed by partial resection of her thymus. Through histology, the calcified tumor exhibited some areas of multilocular fibrous-wall cysts. These cysts were partially lined by small cuboidal cells with severe chronic inflammation and an AB thymoma that arose from the wall of the cyst.
Greater attention should be given to multilocular thymic cysts, to exclude the possibility of neoplasm, especially when the cyst wall is thickened.
Intrahepatic cholangiocarcinoma is a rare malignancy that originates from the epithelial cells of the intrahepatic bile ducts. Intrahepatic cholangiocarcinoma can metastasize in lymphatic chains, including the hepatoduodenal ligament, and it often invades adjacent organs or metastasizes to other visceral organs such as the lungs, bones, adrenal glands, and brain. However, distant skeletal muscle metastasis is very rare. Moreover, a metastatic skeletal muscle tumor rarely shows specific symptoms, making it difficult to identify in a routine examination. A 45-year-old man with a chief complaint of right upper quadrant abdominal pain was admitted to our hospital. Abdominal ultrasound and computed tomography with contrast enhancement showed a malignant mass in the right hepatic lobe, and 2-[18F] fluoro-2-deoxy-D-glucose positron-emission tomography revealed distant skeletal muscle metastases in the thorax and buttock. The patient underwent an ultrasound-guided percutaneous needle biopsy for the metastatic low-echo masses in the skeletal muscle.
Intrahepatic cholangiocarcinoma; Distant metastasis; 18F-FDG PET
The incidence of horseshoe kidney is about 1 in 400 cases. The presence of Wilms' tumor with a horseshoe kidney is unusual, and the occurrence of Wilms' tumor in a horseshoe kidney is estimated at 0.4 to 0.9% of all Wilms' tumors. We report the case of a 5-year-old boy who presented with a stage IV Wilms' tumor in a horseshoe kidney. The patient was treated with preoperative chemotherapy followed by surgical resection and adjuvant chemotherapy. This case illustrates the role of preoperative chemotherapy for preserving renal function and aims to highlight the multimodality treatment of Wilms' tumor.
Adjuvant chemotherapy; Neoadjuvant therapy; Wilms tumor
Serous microcystic adenomas (SMAs), also known as glycogen-rich cystadenomas, are uncommon exocrine tumors mainly involved in the body and tail of the pancreas. In this study, we report two SMA cases of the pancreatic head. In case 1, a 47-year-old woman was referred to our hospital for abdominal bloating and back pain; in case 2, a pancreatic space-occupying lesion was incidentally discovered in an asymptomatic 71-year-old female during an ultrasound. In both cases, a computer tomography scan showed well-demarcated and multilocular cysts in the head of the pancreas, and a central scar and calcifications were noted in case 1. During the exploratory laparotomies, the multiple cystic masses were found in the pancreatic head. A segmental resection of the pancreas and pancreatic jejunal anastomosis were performed. Histologically, the tumors were composed of tiny cysts that were filled with clear fluid and lined by a single layer of cuboidal cells with round, centrally located nuclei and clear cytoplasm, without cellular atypia. Immunohistochemical studies showed that the neoplastic cells were positive for cytokeratin 7 and vimentin but negative for synaptophysin and cytokeratin 20. A postoperative follow-up indicated no recurrence in the patients. We reviewed the literature regarding the etiology, clinical presentation, imaging characteristics, features and management of this lesion.
serous microcystic adenoma; pancreatic head; diagnosis; managements