Leiomyoma of the kidney. Differential diagnostic aspects of renal cell carcinoma with increasing clinical relevance

Renal leiomyoma is a rare benign tumor which has its origin in smooth muscle cells of different structures of the kidney. The clinical incidence of renal leiomyoma is much lower than the frequency described in large autopsy studies. Renal leiomyomas are mainly located in the renal capsula and pelvis or next to those structures. Clinical symptoms are nonspecific (e.g., flank pain or flank tumor) or completely absent, making most of the leiomyomas an incidental finding during routine diagnostics. A differential diagnosis between renal leiomyoma and renal cell carcinoma on the basis of medical imaging is extremely difficult. Therefore, laparotomy and nephrectomy are performed in most cases of suspected renal cell carcinoma. The definitive diagnosis of a leiomyoma is only possible after histological examination of the tumor. Due to the rising number of diagnoses resulting from improved medical imaging, renal leiomyomas are gaining more importance in the differential diagnosis of renal cell carcinoma, especially with respect to kidney-sparing surgery.     

Totally inverted cervix due to a huge prolapsed cervical myoma simulating chronic non-puerperal uterine inversion

INTRODUCTION

Inversion of the uterus is an extremely rare complication of the non-puerperal period and is commonly caused by benign submucous, especially fundal, leiomyomas. A case of a totally inverted cervix due to a prolapsed huge cervical leiomyoma mimicking chronic non-puerperal uterine inversion in a perimenopausal woman is presented.

PRESENTATION OF CASE

A 52-year-old perimenopausal woman was admitted to our clinic with an ulcerated, necrotic, infected and swollen prolapsed mass. Gynecologic history revealed that she was advised myomectomy because of her cervical myoma 2 years ago but she refused to have an operation as she believed that her positive thoughts would shrink the myoma. Presumed diagnosis before surgery was chronic non-puerperal uterine inversion. An intraoperative diagnosis was totally inverted cervix due to a huge cervical leiomyoma. Vaginal hysterectomy without adnexectomy, was performed.

CONCLUSION

This is the first case in the literature which a totally inverted cervix due to a prolapsed huge cervical leiomyoma. Cervical fibroids can grow in perimenopausal period and in extremely rare cases can cause total cervical inversion.     

Epithelioid hemangioendothelioma of the spine presenting as cervical myelopathy: case report

OBJECTIVE AND IMPORTANCE: We report the first case in the literature of cervical myelopathy caused by progressive cord compression as a result of epithelioid hemangioendothelioma of the cervical vertebra. CLINICAL PRESENTATION: A 58-year-old man presented with progressive cervical myelopathy. Imaging revealed a vascular, expansile lesion of contiguous cervical vertebrae causing cord compression. The surgical pathology revealed epithelioid hemangioendothelioma, a rare tumor not previously reported to present in such a fashion. INTERVENTION: Preoperative embolization and a two-stage anterior and posterior surgical decompression and fusion procedure were performed. The high vascularity of this lesion makes surgery a formidable surgical challenge. Adjuvant radiotherapy was administered to the residual tumor because of its potential for low-grade malignancy. CONCLUSION: The diagnosis relied on accurate histopathological assessment. The general principles of achieving cord decompression and tumor control are important. The literature on epithelioid hemangioendothelioma involving the spine is reviewed, and the tumor biology and the role of adjuvant therapy are discussed.     

Cutaneous Leiomyoma with Cytologic Atypia, Akin to Uterine Symplastic Leiomyoma

BACKGROUND: Smooth muscle tumors of the skin with cytologic pleomorphism and mitotic activity have traditionally been characterized as leiomyosarcomas, despite having a benign clinical course. In the uterus, there is a smooth muscle tumor known as symplastic leiomyoma, which typically has cytologic pleomorphism without significant mitotic activity. OBJECTIVE: The objective was to illustrate by case report the unusual histologic variant of the cutaneous pilar leiomyoma analogous to the symplastic leiomyoma of the uterus. METHODS: A 54-year-old woman presented with a clinical picture of cutaneous leiomyoma but had histologic features of nuclear hyperchromasia and pleomorphism, but rare mitoses. RESULTS: Management of this patient included excision of the involved area. CONCLUSION: Symplastic leiomyoma is an atypical uterine leiomyoma with cytologic atypia. We present the case of a patient with cutaneous leiomyomas that histologically demonstrated similar features to a uterine symplastic leiomyoma. We believe that this represents a distinct histologic variant of the cutaneous pilar leiomyoma analogous to the symplastic leiomyoma of the uterus.     

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.     

Symptomatic retro-odontoid pseudotumor causing calcium pyrophosphate dihydrate deposition combined with multilevel cervical spondylotic myelopathy

IntroductionSymptomatic retro-odontoid pseudotumor (ROP) caused by cervical compression and myelopathy is rare. Pathological diagnosis is recommended for differential diagnosis including the following: inflammatory disease, primary bone tumor, metastatic disease and calcium pyrophosphate dihydrate deposition (CPPD) also known as “crowned dens syndrome”. The authors report a rare case of ROP caused by CPPD deposition combined with multilevel cervical spondylotic myelopathy (MCSM) which was treated by tumor resectioning using a transoral approach combined with posterior decompression and fusion.Case presentationA 66-year-old male presented with progressive neck pain and spastic gait with no history of trauma. Radiographic imaging revealed degenerative change involving the atlanto-axial and atlanto-occipital joints with calcified enhancing soft tissue around the odontoid process causing cord compression and cervical instability at the C1-C2 level combined with MCSM and spinal cord compression at C3 to C7. Microscopic assisted transoral tumor resection combined with posterior decompression and fusion was performed at the occiput to T2. The pathology report describes a rhomboid-shaped crystal caused by calcium pyrophosphate dihydrate deposition (CPPD) disease. At the 6-month follow-up following the operation, the patient's neck pain and spastic gait were improved compared to the preoperative examination.DiscussionCervical compression and myelopathy from ROP causing CPPD combined with MCSM is rare. Pathology diagnosis and surgical management are highly recommended.ConclusionIn this case, a combined surgical approach: tumor resection using a transoral approach and a posterior approach for decompression and fusion at occiput to T2 was an effective option for this condition.     

Tetraparesis due to exostotic osteochondroma at upper cervical cord in a patient with multiple exostoses-mental retardation syndrome (Langer-Giedion syndrome)

STUDY DESIGN: Case report of a severe upper cervical cord compression and tetraparesis by a massive cervical exostotic osteochondroma in a patient with multiple exostoses-mental retardation syndrome (Langer-Giedion syndrome; LGS). OBJECTIVE: To describe this very rare pathological condition and the results of surgical intervention. SETTING: Gifu, Japan. METHODS: A 23-year-old man was referred to our clinic because of progressing tetraparesis. He had previously been diagnosed with hereditary multiple exostoses and mental retardation. As he had not complained of any symptoms, his family only noticed the tetraparesis after advanced deterioration. His face possessed the pathognomic features of LGS. A postmyelogram CT scan demonstrated an exostotic mass arising from the left-side C2 pedicle with associated severe spinal cord compression. He was diagnosed with LGS. Hemilaminectomy on the left side and resection of the osteochondroma were performed. RESULTS: At 5 years postoperatively, a neurological examination showed the full return of all motor functions. The CT scan revealed no intracanalar recurrence of the tumor. CONCLUSION: In this case of severe tetraparesis due to cervical osteochondroma, decompression by hemilaminectomy provided excellent results. In patients with LGS and intracanalar osteochondroma, the neurological deficit may be masked by mental retardation. Hence, awareness of this pathological condition will help clinicians diagnose it at an early stage.     

Endobronchial leiomyoma: Report of a case treated by bronchoplasty and a review of the literature

Endobronchial leiomyoma is exteemely rare. Most endobronchial leiomyomas reported in the literature have been resected by either lobectomy or pneumonectomy. We herein report a case treated by sleeve bronchoplasty without pulmonary resection. A 42-year-old woman was admitted to our hospital complaining of hemoptysis. Bronchoscopy revealed a lobulated tumor arising from the medial wall of the right main stem bronchus. A sleeve resection of the right main bronchus including the tumor and end-to-end anastomosis was performed. The histological diagnosis of the resected specimen was leiomyoma with no evidence of malignancy. The importance of early diagnosis and appropriate surgical treatment to preserve pulmonary function are emphasized. Similar cases of an endobronchial type of pulmonary leiomyoma reported in the literature are also reviewed.     

Benign metastasizing leiomyoma: metastasis to rib and vertebra

Benign metastasizing leiomyoma is very rare and characterized by the presence of pelvic, peritoneal, nodal, or pulmonary nodules in women with a history of uterine leiomyomas. We report a case of benign metastasizing leiomyoma in a 30-year-old woman who had undergone a prior myomectomy due to uterine cellular leiomyoma 3 years earlier. The patient had a mass on the right sixth rib and 2 masses in the sixth thoracic vertebra. Pathologically, these masses were diagnosed as cellular leiomyomas. Estrogen and progesterone receptors were both positive in the metastatic tumors as well as in the uterine leiomyomas. The diagnosis of benign metastasizing leiomyoma can only be made after careful examination of the primary tumor to exclude small foci of malignant change.     

Cervical radiculopathy secondary to Hodgkin's lymphoma

BACKGROUND: Spinal cord/root compression is a rare complication of HL and usually seen in the setting of progressive, advanced disease. It is extremely rare to occur as an initial presentation of HL. We report a case of HL presented with bilateral cervical radiculopathy. METHODS: A case report of a 42-year-old woman who presented with C8 cervical radiculopathy and paraspinal mass with extradural extension in C7-T1. Biopsy revealed HL. This case report represents a case of HL presenting with epidural disease, with nerve root compression as the only apparent site of clinical involvement. A review of the literature of patients with HL presenting with spinal cord/root compression is presented. RESULT: Our patient received chemotherapy and external beam radiation therapy. She achieved good recovery of her symptoms and complete response by radiologic criteria. Based on a review of the literature, Hodgkin's disease involving the spinal epidural space is very responsive to chemoradiotherapy with good prognosis for both functional recovery and complete response. CONCLUSION: Chemoradiotherapy is a successful treatment for Hodgkin's disease presenting with spinal root compression. Surgery should be reserved for urgent decompression, if needed; palliation; and maintenance of function and stability.     

Pelvic retroperitoneal leiomyoma

Primary retroperitoneal leiomyomas are a rare and unusual differential diagnosis in the acute abdomen. A 26-year-old woman presented with chronic constipation and acute abdominal pain. The diagnostic workup included contrast computer tomography, transabdominal ultrasonography, and colonoscopy that revealed an 8-cm homogeneous pelvic tumor with rectal compression. Retroperitoneal leiomyoma was suspected on imaging studies and finally confirmed after tumor excision and immunohistochemical staining.     

Spinal Glioblastoma Multiforme: Unusual Cause of Post-Traumatic Tetraparesis

Abstract

Background/Objective: Glioblastoma multiforme (GBM) is the most common glial cell tumor of the adult brain. However, primary GBM of the spinal cord is a rare condition.

Methods: Case report.

Results: A young man presented with acute onset quadriparesis after a whiplash injury. A magnetic resonance scan showed the typical appearance of a high-grade intramedullary tumor with fusiform expansion of the entire cervical cord. Subtotal decompression and biopsy was done by posterior laminectomy, followed by external beam radiotherapy. Signs and symptoms improved after the completion of radiotherapy but did not resolve completely. Death caused by respiratory failure occurred 3 months later. Conclusions: This presentation of GBM of the cervical cord is rare; an intramedullary tumor should be considered when minor cervical trauma results in disproportionate neurologic deficit. To the best of our knowledge, this is the first reported case of spinal GBM with extensive pan-cervical involvement.     

Successful surgical treatment of a giant uterine leiomyoma: A case report

Introduction and importanceUterine leiomyoma is a common disease. The tumor gradually increases and becomes a target for treatment when accompanied by certain symptoms. It rarely grows into a giant uterine leiomyoma, which is defined as leiomyoma weighing >11.34 kg.Case presentationA 58-year-old Japanese woman had a history of putamen hemorrhage and deep vein thrombosis. A giant uterine leiomyoma prevented her from walking, and she scheduled surgery for its removal. The tumor was 46 × 35 × 27 cm, and the uterine arteries and veins were extremely dilated. A blocking balloon catheter was placed in the abdominal aorta to prevent massive bleeding, and a filter was placed in the inferior vena cava to prevent pulmonary thromboembolism. The surgery focused on careful vascular treatment, with selective ligation of the ovarian arteries and veins and the uterine arteries. The total amount of bleeding was 1130 g, and the uterus was removed without complications. The weight of the excised tissue was 22.6 kg.Clinical discussionSurgical treatment of the largest giant uterine leiomyomas is rare and challenging. Previous reports addressed the risk of massive bleeding and perioperative death. Surgery is the best treatment for giant uterine leiomyomas, but perioperative management and surgical procedures require complex and elaborate planning.ConclusionVery few gynecologists have experience treating giant uterine leiomyomas. Successful surgery requires careful surgical preparation, and the gynecological oncologist must have extensive experience with giant leiomyomas.     

beta(2)-Microglobulin amyloidosis caused spinal cord compression in a long-term haemodialysis patient

STUDY DESIGN: A case report of cervical myelopathy caused by epidural beta (2)-microglobulin (beta2m) amyloid deposits in a 50-year-old woman with haemodialysis treatment. OBJECTIVE: Long-term haemodialysis in patients with end-stage renal disease leads to several complications based on beta2m deposits, which can affect, in the cervical spine, the intervertebral disk, and in rare cases, they may compress the spinal cord and nerves. The objective of this report is to describe the clinical and radiological follow-up preceding the indispensable surgical excision of an amyloid mass in a 50-year-old woman with haemodialysis treatment. Long-term postoperative cervicalgia owing to subcondylian bone cyst-associated atlanto-occipital instability is also described and discussed. SETTING: Department of Neurosurgery A, Hop Pellegrin, Bordeaux, France. CASE REPORT: We present a clinical case of a patient with spinal cord compression. The patient was treated by surgical excision of an amyloid mass subsequent to a C2-C3 laminectomy. The patient experienced clinical improvement with a regression of all of her neurological symptoms. Histological findings confirm the diagnosis of beta2m amyloid deposition. However, 5 years after surgery the subcondylian bone cysts were still observed and atlanto-occipital instability required her to wear a minerva. CONCLUSION: Our case report confirms that surgical excision of beta2m epidural deposits is necessary and relevant when neurological prognosis is discussed, and that pain is still the major symptom of disease evolution. The use of high-flux synthetic membranes could decrease the beta2m blood level and early renal graft is the only method to prevent such complications.     

Leiomyoma of Testis –A Case Report

Leiomyomas, benign tumors, can arise from any organ containing smooth muscle. In the genitourinary tract, the renal capsule is most commonly involved. Intrascrotal leiomyomas are infrequently seen. Testis-associated leiomyomas are extremely rare. We report here testis associated leiomyoma.     

Leiomyomas of the female urethra and bladder: a report of five cases and review of the literature

Through the experience of five cases of leiomyoma developed in the female bladder and urethra with a review of the literature, we have made an effort to characterize the association of symptom with the size and location of the tumor and demonstrate an appropriate treatment. The study population was composed of patients who underwent surgery for bladder or urethral leiomyoma in our hospital from March 1990 to April 2005. Their medical records were reviewed retrospectively concerning the symptom, size and location of leiomyoma, the result of cystoscope and radiological examination, surgical method, pathologic report, complications, and recurrence. Four cases were diagnosed as urethral leiomyoma and one case as bladder leiomyoma. All patients with urethral leiomyoma were admitted for the chief complaint of a palpable tumor. When the tumor size was small, if it was located on the lateral side of the urethra, it was asymptomatic, but if located in the midline, it presented irritative or obstructive symptom. When it was big, if located on the lateral side, it presented irritative rather than obstructive symptom, and if located in the midline, it presented obstructive symptom. One case of bladder leiomyoma was discovered incidentally during ultrasonic exam. In all five cases, surgical removal was performed and complications or recurrence were not detected afterwards. Bladder and urethral leiomyomas are very rare and cause diverse manifestations from asymptomatic to irritative or obstructive symptom. It is presumed that the location and size of the tumor are associated with symptom. Unless it is the case with severe hemorrhage or obstructive acute renal failure, immediate surgery is not required. However, it is desirable to distinguish leiomyoma from malignant or other benign tumors by surgical biopsy or removal.     

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.     

Ureteral endometriosis and coexistent urethral leiomyoma in a postmenopausal woman

We report the case of a postmenopausal woman with a synchronous obstructing intrinsic endometrioma of the left ureter and a coexistent periurethral leiomyoma. Endometriosis in postmenopausal women is a rare clinical entity usually associated with exogenous estrogen use. Urethral leiomyomas are also rare, with only 40 cases reported in the literature. Ovarian hormones are believed to influence the growth of leiomyomas. We report the genitourinary presentation of 2 separate disease entities with known hormonal influence in a postmenopausal woman receiving estrogen replacement therapy. We believe the patient's hormonal milieu affected the development of her concurrent pathology.     

Removal of an anterior spinal dermoid cyst with fenestra corpectomy in Klippel-Feil syndrome: technical case report

OBJECTIVE AND IMPORTANCE: A spinal cord tumor occurring in association with Klippel-Feil syndrome is quite rare. The removal of an anteriorly located spinal cord tumor at the level of block vertebrae creates a surgical challenge. CLINICAL PRESENTATION: A case of an intradural extramedullary dermoid cyst located anterior to the spinal cord and a syringomyelic cavity at the level of block vertebrae in a 43-year-old woman with Klippel-Feil syndrome is presented. She experienced pain and numbness in both shoulders and in her neck, and she had a slight weakness in both arms before the operation. Her weakness and the clinical symptoms completely disappeared after the operation, and the resolution of the syringomyelic cavity was observed at control magnetic resonance imaging. INTERVENTION: An anterior approach creating a fenestra corpectomy to the block vertebrae was performed, and the tumor was removed totally. No fusion or fixation was performed. CONCLUSION: To our knowledge, this is the first report of an anteriorly located intradural extramedullary cervical spine tumor in association with Klippel-Feil syndrome treated with this surgical technique. A three-dimensional computed tomographic control scan obtained 1 year after the operation did not show any instability.