Renal leiomyoma: Case report and literature review

Renal leiomyomas are rare benign tumours of the kidney originating from muscle cells. They are usually found by an autopsy, whether the patient is asymptomatic or has symptoms (i.e., abdominal/flank pain, hematuria, palpable mass). Today the widespread use of ultrasonography and computed tomography has increased the detection of clinically asymptomatic renal leiomyomas. The differential diagnosis between leiomyomas and other malignant lesions (above all renal cell carcinoma or leiomyosarcoma) is still possible by histological examination. Radiological examinations are not sufficient for the differential diagnosis. Renal leiomyomas have no aggressive behaviour and they usually do not metastasize. The prognosis, after surgery, is excellent without recurrence. We report a case of leiomyoma in a 31-year-old man who presented hematuria and flank pain. We also review the literature and provide a summary of clinical, radiological and histological features of renal leiomyomas.     

Leiomyoma of the kidney: presentation of 4 new cases and the role of computerized tomography

Renal leiomyoma is a challenging diagnostic and therapeutic condition. It is clinically similar in presentation and radiographic appearance to its malignant counterpart, leiomyosarcoma. We review 30 cases of clinically diagnosed leiomyoma of the kidney from the literature, including 4 new cases with emphasis on the computerized tomography findings. Computerized tomography may locate a renal leiomyoma serendipitously in an asymptomatic patient. If the lesion is peripheral or in the parapelvic area and a plane can be seen between the tumor and kidney a capsular tumor, such as a leiomyoma of the kidney, might be considered in addition to the more common renal cell carcinoma. Renal leiomyomas have a variable radiographic pattern from that of a pure cystic to a mixed solid/cystic to an entirely solid lesion. Renal leiomyomas usually are sharply demarcated from the surroundings. Although computerized tomography cannot distinguish a renal leiomyoma from other benign or malignant renal processes the presence of invasion can virtually eliminate this benign tumor as a diagnosis. Since a preoperative diagnosis cannot be made, management involves renal exploration and radical nephrectomy in the larger lesions with a renal-sparing operation possible in selected cases. After treatment patients have a uniformly excellent prognosis.     

Giant renal leiomyoma: a case report and brief review of the literature

We report on a very large renal leiomyoma in a man presenting with a 1-year history of lower back and flank pain and a rapidly growing abdominal mass. Since a cystic renal cell carcinoma could not be ruled out, a postembolization right radical nephrectomy was performed. Diagnosis was confirmed by pathologic and histologic analysis. Renal leiomyomas are very rare benign tumours that are nearly indistinguishable from leiomyosarcoma or renal cell carcinoma preoperatively. This case represents the second largest such entity reported and demonstrates the limited ability of accurate diagnostic determination preoperatively, with pathologic examination and immune-histochemical staining postnephrectomy representing the only definitive means of diagnosis. A brief review of the literature and an outline of typical clinical and pathologic features of renal leiomyomas are also presented.     

Two cases of leiomyoma of the kidney]

Renal leiomyomas large enough to be clinically diagnosed are extremely rare. We review 30 cases of clinically diagnosed renal leiomyoma from the literature in Japan, including our 2 new cases. Case 1: In a 52-year-old man with no symptoms a renal mass was found accidentally on an ultrasonogram. CT scan showed a mass with a cystic area at the upper pole of the right kidney. Angiogram showed a hypovascular mass. Case 2: CT scan revealed a cystic mass and angiogram showed an avascular mass at the upper pole of the left kidney in a 19-year-old man having gross hematuria and left flank pain. Transperitoneal nephrectomy was done in both cases. Histologically each tumor was composed of monotonous proliferation of spindle shaped cells without atypia, which showed intense immunoreactivity for alpha-smooth muscle actin. The diagnosis of benign leiomyoma was made in each case. In a review of 30 cases, we found that renal leiomyomas occur most often in female (77%), between decades 2 and 5 of life (median: 46 years). On the angiogram it appears most often as an avascular or hypovascular mass. CT scan shows cystic or mixed solid/cystic or solid lesion, occasionally with calcification. Preoperative diagnosis is extremely difficult to be made. Histologically, fibroma, angiomyolipoma, congenital mesoblastic nephroma and leiomyosarcoma should be differentiated.     

Renal capsular leiomyoma

An exophytic renal mass was diagnosed preoperatively as renal cell carcinoma by ultrasound, computerized tomography and arteriography. Intraoperative recognition of the proper diagnosis of leiomyoma of the renal capsule permitted renal-sparing excision. Renal capsular leiomyoma should always be considered in the differential diagnosis of an apparent exophytic renal cell carcinoma.     

Renal cell carcinoma in Japanese children

Renal cell carcinoma in children is extremely rare compared to Wilms tumor. We report on a 7-year-old Japanese boy with left renal cell carcinoma. Since the disease was clinical stage I, nephrectomy with tumor extirpation was performed after a short course of initial chemotherapy had been administered. The patient is free of disease 3 years postoperatively. In a review of the Japanese literature we found 71 reports of renal cell carcinoma in children less than 15 years old. Mean patient age at occurrence was 8.1 years, there were no differences in regard to the sex or the affected side, and the most frequent symptom was the presence of a mass. We stress the early establishment of diagnosis in children with a persistent abdominal mass, hematuria and flank pain, since surgical treatment leads to a favorable prognosis only in the early stage of renal cell carcinoma.     

Surgical enucleation for renal cell carcinoma. A case report

We present a case of bilateral synchronous renal cell carcinoma treated with surgical enucleation. Urological consultation was asked to evaluate masses of the kidneys, which were detected during a diagnostic imaging on a 58-year-old man with hepatic disorder. Excretory urograms showed definite bilateral upper pole renal masses. Bilateral selective renal angiograms disclosed neovascularity in the small masses. Surgical enucleation of the left renal tumor by a flank approach was performed. The surgical specimen being 3.5 by 2.5 by 2 cm (29 g) was pseudoencapsulated and was identified renal cell carcinoma of the clear cell type, grade 1. INF alpha, pathologically. Seven weeks after, a pseudoencapsulated tumor of 3 by 2.5 by 2 cm (16 g) in the right upper pole was removed by simple enucleation. The pathological diagnosis was renal cell carcinoma of clear cell type, grade 2, INF alpha. The patient is well without evidence of recurrent or any residual disease at 21 months after the second operation. Renal function remains with in normal limits (CCr 110 ml/min). Of our collected cases of bilateral synchronous renal carcinomas treated by bilateral conservative surgery, clinical data are available for 11. Including our case, a total of 12 cases are reviewed.     

Hereditary leiomyomatosis and renal cell cancer: a syndrome associated with an aggressive form of inherited renal cancer

PURPOSE: Hereditary leiomyomatosis and renal cell cancer is a recently described hereditary cancer syndrome in which affected individuals are at risk for cutaneous and uterine leiomyomas, and kidney cancer. Our initial experience revealed the aggressive behavior of these renal tumors, often with early metastasis, despite small primary tumor size. We report the clinical characteristics and urological treatment of patients with hereditary leiomyomatosis and renal cell cancer associated renal tumors. MATERIALS AND METHODS: A total of 19 patients with hereditary leiomyomatosis and renal cell cancer associated renal tumors were evaluated. The 11 women and 8 men had a median age at diagnosis of 39 years (range 22 to 67), and a median clinical and radiological followup of 34 months (range 6 to 141). Hereditary leiomyomatosis and renal cell cancer manifestations in patients with renal tumors included cutaneous leiomyomas in 11 of 17 evaluable patients (65%) and uterine leiomyomas in 7 of 7 evaluable females (100%). RESULTS: Median pathological tumor size was 7.8 cm (range 1.5 to 20). Histological subtypes were consistent with hereditary leiomyomatosis and renal cell cancer renal carcinoma. Four of 7 patients with 2.0 to 6.7 cm T1 tumors had spread to regional lymph nodes or metastases at nephrectomy. Overall 9 of 19 patients (47%) presented with nodal or distant metastases. CONCLUSIONS: Renal tumors in patients with hereditary leiomyomatosis and renal cell cancer syndrome are significantly more aggressive than those in patients with other hereditary renal tumor syndromes. In contrast to other familial renal cancer syndromes, the observation of 3 cm or less renal tumors associated with hereditary leiomyomatosis and renal cell cancer is not recommended. Careful followup of affected and at risk individuals in families is necessary.     

Renal cell carcinoma: case report and literature review

Renal cell carcinoma arising from epithelial cells of the renal tubule is a highly aggressive and malignant tumor in all ages. Less than 2% of cases occur in childhood, relatively in older age group. Only a few pediatric series have been presented in the English literature. Tumor is presented with characteristic findings of flank pain, gross hematuria, and palpable mass. Although one half of the patients have metastasis at the time of diagnosis, most cases are currently being incidentally detected using improved imaging techniques. The overall prognosis in children appears to be similar to that in adults. Tumor stage and complete surgical resection have been reported as the most meaningful prognostic factors for the outcome. The incidence of metastatic disease is same as in adults. The effect of chemotherapy and immunotherapy, either preoperatively or postoperatively, is unclear. Cure is the most likely consequence in localized and completely resected tumors. Here, we present an 8-year-old boy with renal cell carcinoma demonstrating only hematuria without any pathological physical examination findings. The mass was described by abdominal ultrasonography and computed tomography in the left kidney. After the left nephroureterectomy, the patient was given no therapy.     

Renal cell carcinoma with widespread metastasis in 19 years old young male – A rare case report

Introduction and importanceRenal cell carcinoma is the most common malignant tumor of the kidney which occurs more frequently in men and older people than in women and young adults. Renal cell carcinoma is the second most common renal malignancy diagnosed among pediatric and adolescent patients comprising of 2% to 6% of renal cancers.Case presentationA 19 years old young adult male came with a history of epigastric and back pain, hematuria and weight loss. Per abdominal examination showed a palpable mass in the epigastric and left hypochondriac region. Radiological imaging showed diffuse infiltration of renal interstitium with multiple hypodense lesions in left kidney, renal vein infiltration, and lytic destruction of vertebral bodies and left superior pubic ramus. Fine needle aspiration cytology and trucut biopsy was taken which confirmed renal cell carcinoma, clear cell type with bone metastasis.Clinical discussionAlthough most renal cell carcinoma is sporadic and relatively uncommon in young adults, the incidence of renal cell carcinoma in this age group has steadily increased. Young adults are less likely to receive diagnosis of renal cell carcinoma incidentally. A few reported pediatric series have shown that renal cell carcinoma is highly aggressive, tends to be invasive, and metastasizes to the lungs and bones.ConclusionYoung adult with clear cell renal cell carcinoma showing wide spread metastasis is rare. Since, young age is an independent prognostic factor for cancer-specific survival, early diagnosis of the tumor will be beneficial for patients.     

Large renal leiomyoma

Renal leiomyomas are uncommon between renal tumors, and they have a benign mesenchymatous origin. Because of their low incidence, unspecific symptomatology an not well-defined iconographic semiology, they often raise problems with differential diagnosis from kidney masses, although they are often big size lessions. We report a patients with incidentally diagnosis of big solid left renal mass, who underwent radical nephrectomy resulting kidney pelvis leiomyoma. Diagnostic, histological and therapeutic aspects are briefly review in literature. Emphasis is made on the relevance of a high index suspicion considering big solid asymptomatic renal masses in middle-age women. Specially in renal tumors with well-defined limits and abscence of locoregional and systemic dissemination. We emphasized usefulness of Magnetic Angioresonance, immunohystochemical test and conservative surgery opportunity in small renal leiomyomas preoperatively confirmed.     

Variants of renal angiomyolipoma closely simulating renal cell carcinoma: difficulties in the histological diagnosis

Renal angiomyolipoma is considered to be a benign renal tumor composed of atypical blood vessels, smooth muscles and fat cells. We report 2 cases of unilateral renal angiomyolipoma. In both cases, our preoperative diagnosis was renal cell carcinoma, because no low density area compatible with fat tissue was noted in the tumors on radiographic evaluation. Through histological examination, both tumors proved to be angiomyolipomas mainly composed of epithelioid cells in 1 case, and spindle-shaped smooth muscle cells mimicking a leiomyoma in the other case. Both patients are well showing no evidence of metastases 16 and 14 months after nephrectomy, respectively.     

肾海绵状血管瘤的诊断和治疗（附3例报告）

目的：探讨肾海绵状血管瘤的诊断与治疗。方法：回顾性分析3例肾海绵状血管瘤患者,男2例,女1例,年龄17-34岁。临床表现包括腰痛1例,肉眼血尿1例,腰痛并镜下血尿1例。多普勒超声和CT扫描显示肾脏肿块,2例体积较大,其中1例伴有肾静脉血栓形成。2例男性患者行肾切除,1例女性患者行肿块切除。结果：3例患者术后血尿,腰痛均消失。术后随访6个月-2年,无复发或并发症,复查CT和尿液检查未见异常。结论：肾海绵状血管瘤是一种罕见的良性病变,其症状主要为腰痛、血尿。肾海绵状血管瘤少有术前诊断,多为术后病检证实,CT、B超、MRI和肾血管造影均有诊断价值,但不能完全与恶性肿瘤区分。手术治疗是主要的治疗方法。     

Inflammatory pseudotumor of the kidney

Renal inflammatory pseudotumor is a very rare benign condition of unknown etiology characterized by proliferative myofibroblasts, fibroblasts, histiocytes, and plasma cells. We report a case of inflammatory pseudotumor of the kidney. A 42-year-old female was admitted with the complaint of right flank pain. Computerized tomography revealed a tumor, 7 cm in diameter, in the upper pole of the right kidney. Right radical nephrectomy was performed under the diagnosis of renal cell carcinoma. Histological examination demonstrated a tumor composed of spindle-shaped fibroblastic cells infiltrated by variable numbers of plasma cells, small lymphocytes and histiocytes. The pathologic diagnosis was renal inflammotory pseudotumor.     

Benign metastasizing leiomyoma causing spinal cord compression

BACKGROUND: Leiomyoma as a cause of cord compression is extremely rare. To our knowledge this is the first report of a dural-based leiomyoma and second report of a leiomyoma causing cord compression. CASE DESCRIPTION: A 38-year-old female renal transplant recipient presented with features of cervical cord compression. On imaging and at surgery, the tumor was mistaken for a neurofibroma. A cervical laminectomy and near total excision of the tumor was done. She did well in the postoperative period but presented 5 months later with thoracic empyema leading to septicemia and her demise. Ultrasound examination and autopsy showed leiomyomas in many other sites including the uterus. CONCLUSIONS: The presence of extrauterine and uterine leiomyomas is a curious condition termed as "benign metastasizing leiomyoma." As our patient was on immunosuppressant therapy following a renal transplant, it might have predisposed her to this rare condition with multifocal tumors.     

Leiomyoma of the genitourinary tract.

OBJECTIVE: To review our experience and the literature with respect to leiomyomas of the genitourinary tract with special emphasis on the role of the clinical presentation and imaging studies in the differential diagnosis. MATERIAL AND METHODS: During a period of 35 years in our department, five patients were treated with symptomatic leiomyomas, originating from the renal pelvis, bladder, urethra and epididymis. All the cases were diagnosed after exploration, excision and histological examination of the specimen. Following the presentation of the five cases of leiomyomas of the genitourinary tract, we reviewed the literature with special emphasis on the modern imaging techniques, differential diagnosis and treatment approach. CONCLUSION: Complete excision followed by histological examination is the most reliable means of distinguishing leiomyoma from other more common and usually malignant tumors of the genitourinary tract.     

肾髓质癌1例报告及文献复习

目的:探讨起源于肾盏上皮的肾髓质癌,分析其临床、病理形态学、影像学、鉴别诊断及分子生物学特征,以提高本病的诊疗水平。方法:对最近收治的1例肾髓质癌患者的诊治过程进行回顾性分析,结合国内外最新的文献对本病的临床、病理学、影像学、鉴别诊断及分子生物学等特征以及治疗和预后进行总结分析。结果:本病好发于中青年,伴有镰状细胞病(Sickle cell disease,SCD)或镰状红细胞特征是其主要特点。肿瘤生长于肾髓质内;瘤细胞呈片块状弥漫分布,网状或巢状排列,间质明显纤维化及许多中性粒细胞浸润形成微脓肿是其特征性的变化。与其他肾肿瘤相比,影像学上没有明显的特异表现。CK、Vim和EMA均+,有助于同肾集合管癌相鉴别。拓扑异构酶2α有望成为肾髓质癌治疗的新靶点。结论:肾髓质癌是一种少见的侵袭性恶性肿瘤,影像学上无特异性,确诊主要靠病理诊断,主要应与肾集合管癌相鉴别。探索该病的新靶点,尽量早期诊断治疗,是提高其疗效的关键。     

A giant cystic leiomyoma mimicking an ovarian malignancy

INTRODUCTIONLeiomyoma of the uterus is the most common type of tumor affecting the female pelvis and arises from uterine smooth muscle. The size of leiomyomas varies from microscopic to giant; giant myomas are exceedingly rare. We report an unusual case of a large, cystic, pedunculated uterine leiomyoma mimicking a primary malignant ovarian tumor on sonography and CT.PRESENTATION OF CASEA 58-year-old postmenopausal nulliparous woman presented with a history of lower abdominal pain and distension for a period of approximately 12 months. The patient's personal history revealed difficulty in walking, tiredness and recent weight gain of approximately 25 kg. Sonography and CT examination showed a large mass that filled the abdomen. A preoperative diagnosis of a primary malignant ovarian tumor was made. The patient underwent laparotomy, total hysterectomy and bilateral salpingo-ooferectomy. The histology revealed a leiomyoma with extensive cystic degeneration.DISCUSSIONThe current established management of uterine fibroids may involve expectant, surgical, or medical management or uterine artery embolization or a combination of these treatments. A surgical approach is preferred for management of giant leiomyomas.CONCLUSIONPedunculated leiomyomas should be considered in the differential diagnosis of a multilocular and predominantly cystic adnexal mass.     

A case of xp11.2 translocation renal cell carcinoma

Xp11.2/TFE3 translocation renal cell carcinoma (RCC), a recently classified distinct subtype, is a rare tumor that usually affects children and adolescents. The morphology and biological behavior are not widely recognized, Xp11.2 translocation RCC is suggestive of early metastases despite the small tumor size. The definitive diagnosis requires the evidence of several different reciprocal translocations involving the TFE3 gene located on chromosome Xp11.2. Here, we present a case of Xp11.2 translocation RCC in an 18-yearold male. He was referred to our hospital because of a right renal tumor with macroscopic hematuria and right flank colic. The radiographic evaluation including magnetic resonance imaging (MRI) suggested it to be a typical papillary renal cell carcinoma or benign renal tumor. He underwent laparoscopic nephrectomy against the repeat symptom in spite of small tumor (3.5 cm in diameter). The immunohistochemical study revealed nuclear staining for TFE3 protein in the cancer cells. The urologic and radiologic outcomes were satisfactory after more than 1 year of follow-up.     

肾髓质癌1例报告及文献复习

目的:探讨起源于肾盏上皮的肾髓质癌,分析其临床,病理形态学,影像学,鉴别诊断及分子生物学特征,以提高该病的诊疗水平。方法:对1例肾髓质癌的诊治过程进行回顾性分析,结合国内外文献对本病的临床,病理学,影像学,鉴别诊断及分子生物学等特征以及治疗和预后进行总结分析。结果:本病好发于中青年,伴有镰状细胞病或镰状红细胞特征是其主要特点。与其他肾肿瘤相比影像学上没有明显的特异表现。CK、Vim和EMA均(+)有助于同肾集合管癌相鉴别。拓扑异构酶2α有望成为肾髓质癌治疗的新靶点。结论:肾髓质癌是一种少见的侵袭性恶性肿瘤,影像学上无特异性,确诊主要靠病理诊断,主要应与肾集合管癌鉴别。探索该病的新靶点,尽量早期诊断治疗是提高其疗效的关键。