Bilateral adrenal tumor: causes and clinical features in eighteen cases

Bilateral adrenal neoplasms are very rare. Studies have shown that most are metastatic tumors, and clinical presentation varies with tumor type. We retrospectively reviewed medical records of 18 cases of bilateral adrenal tumor in our hospital between 2002 and 2007. The etiology was pheochromocytoma in six, primary lymphoma in four, nonfunctioning cortical adenoma in four, metastatic tumors in two, primary aldosteronism in one, and Cushing syndrome in one. Patients with lymphoma had largest average tumor size. Our findings suggest that pheochromocytoma, primary lymphoma, and nonfunctioning cortical adenoma are common causes of bilateral adrenal tumor.     

Accuracy of frozen section diagnosis of the salivary gland

Three hundred and one salivary gland lesions (162 benign, 72 malignant, and 67 benign non-neoplastic) of 677 cases were evaluated by use of intraoperative frozen sections by 66 pathologists. In seven patients, the diagnosis was deferred for permanent sections. In four cases (1.3%), the diagnosis at permanent section changed from one category of benign tumor to another, and in five cases (1.7%), from one category of malignant tumor to another. In four tumors, a frozen section diagnosis of benign was changed to malignant on permanent sectioning; all four involved acinic cell carcinomas. Only two tumors were incorrectly diagnosed as malignant. We conclude that diagnoses of most salivary gland lesions based on frozen section examination are reliable and accurate. However, the literature does indicate that caution should be exercised when malignant tumors are dealt with.     

Tubular Krukenberg tumor. A problem in histopathologic diagnosis

A review of a series of 70 Krukenberg tumors seen in consultation disclosed 13 cases with a predominant tubular pattern. Eleven of them had been diagnosed by the referring pathologist as a tumor in the sex cord-stromal category, usually a Sertoli-Leydig cell tumor; no diagnosis was preferred in the other two cases. Three factors contributed to the erroneous diagnoses: a prominent tubular pattern, luteinization of the stroma of the tumor in five cases, and associated virilization in two cases. Each tumor, however, contained typical signet-ring cells that were readily demonstrable with mucicarmine stains. In six cases the tumors were unilateral and in seven, bilateral. Ten patients died of their cancer from 2 to 21 months after the diagnosis had been made. In one case the ovarian tumors were not discovered until autopsy. Two patients are alive 7 and 9 months postoperatively. A primary tumor was found in the stomach in four cases and in the sigmoid colon and appendix in one each. No primary tumor was found in seven cases but an autopsy had been performed in only one of these. The diagnosis of Krukenberg tumor must always be considered in the differential diagnosis of an ovarian tumor with a tubular pattern even though endocrine manifestations are present.     

Co-existence of renal cyst and tumor: report of two cases and a review of the literature

We report on two patients with renal cyst and tumor in the same kidney. Despite the diagnosis of benign renal cyst obtained by radiological investigation, one case proved to be malignant at surgical exploration. The other patient was diagnosed to have renal cyst and tumor in the same kidney preoperatively. Co-existence of renal cyst and tumor is considerably rare. Thirty four cases collected from the Japanese literature, including our two cases, are reviewed and discussed.     

The nonopacified kidney with Wilms' tumor

We reviewed 56 cases in which the preoperative diagnosis was Wilms' tumor at the Children's Hospital of Philadelphia (CHOP) during the three calendar years 1983 through 1985. Fifty-one had Wilms' tumor (38 operated on at CHOP; 13 were referrals for further treatment or consultation), three had renal cell carcinoma (RCC), and one each with metastatic mesoblastic nephroma and clear cell sarcoma. We reviewed these cases to determine the significance of the urographic finding of nonopacification of kidney with suspected Wilms' tumor, and what additional diagnostic studies are needed. Five Wilms' tumor patients (10%) and one RCC patient had nonopacification of the involved kidney on intravenous pyelography (IVP). Four Wilms' tumor patients had tumor extending into the collecting system: into the proximal ureter in one, obstructing the superior pole in another, and filling the renal pelvis in two, one of which also had tumor extending through the inferior vena cava and into the right atrium. One patient with Wilms' tumor and one with RCC had huge left-sided tumors that crossed the midline and appeared to replace completely the functioning renal parenchyma on that side. The most common cause of nonpacification of kidney by Wilms' tumor is collecting system blockage by tumor. Although extension into the renal pelvis is relatively uncommon in Wilms' tumor (five of 51 cases during the study period, 10%), when it occurs, nonopacification of IVP often results (four of five cases, 80%). Although renal vein involvement is more frequent (eight of 51 cases; 16%), nonopacification is unusual (one of eight cases, 13%; the tumor also had filled the collecting system).(ABSTRACT TRUNCATED AT 250 WORDS)     

Long-term efficacy of percutaneous drill-biopsy under computed tomography guidance of osteoid osteomas of the hip and femur. A review of seven cases

En bloc surgical excision of osteoid osteomas of the hip and femur is difficult because of problems with tumor boundary identification and the need to use internal fixation or bone grafting. We report our experience with seven cases treated by percutaneous drill-biopsy under computed tomography guidance. PATIENTS: Six adults and one child with a mean age of 22 years treated since 1995. All seven patients reported insomnia due to pain during a mean of 10 months, improved by aspirin or other nonsteroidal anti-inflammatory agents. The tumor was seen on plain radiographs in four cases and on computed tomography scans in all seven cases. Magnetic resonance imaging was diagnostic in only two cases. The tumor was in the femoral neck in four patients, in the acetabulum in one, in the proximal femur in one, and at the distal diaphyseal-metaphyseal junction of the femur in one. METHOD: A trephine was used to remove the tumor under computed tomography guidance during a short general anesthesia. RESULTS: No serious complications were recorded. Full weight bearing was allowed starting at the forty-eighth hour in six of the seven patients. Immediate pain relief and a full recovery were obtained in every case. Results were excellent in four patients and good in three after a follow-up of 14 to 44 months. CONCLUSION: Percutaneous drill-biopsy of osteoid osteomas is a valuable alternative to conventional surgery in patients with tumors at sites that are difficult to access. The technique allows early weight bearing and ensures a full recovery.     

Genital Buschke Loewenstein tumors. Report of 4 cases

The authors present four cases of Buschke Loewenstein tumor. In three cases, the outcome was good despite areas of anaplasic change are seen in one of them. The outcome of the fourth patient was fatal because of multiples recurrences. Clinical features, diagnosis and treatment of this rare disease are discussed.     

囊性肾癌5例报告并文献复习

目的:探讨囊性肾癌的早期诊断及治疗方法。方法:回顾性分析2008年1月~2012年8月间收治的5例囊性肾癌患者的临床资料,1例术前影像学检查提示双侧肾脏占位病变,一侧为囊性占位病变;4例显示单侧肾脏单发囊性占位病变,左肾3例,右肾1例;其中男3例,女2例;年龄46~68岁,平均56.4岁;肿瘤体积21.84~208ml,平均101.96ml。按照Bosniak分类,Ⅱ类1例,Ⅲ类2例,Ⅳ类2例;T1期2例,T2期2例,T3期1例。3例术前诊断为囊性肾癌,1例为肾脏囊肿,1例为肾嗜酸性细胞瘤合并囊性肾癌。2例行肾癌根治术,2例行肾部分切除术,1例行囊肿去顶减压术后2周行肾癌根治术。并结合相关文献复习进行分析讨论。结果:5例患者术后病理检查报告证实为透明细胞癌2例,乳头状细胞癌1例,多房囊性肾癌1例,嗜酸细胞腺瘤伴囊性变1例。平均随访34.4个月(10~66个月),1例发生骨骼及肺部转移,余4例均无复发转移。结论:囊性肾癌是一种广义上的肾癌分类,有四种分型;与其他类型肾癌相比,大部分恶性程度较低。囊性肾癌的术前诊断主要依赖于影像学检查,对于可疑病例,术中需行快速冷冻病理检查。对此类肿瘤,建议行保留肾单位手术。     

Management of thymic cysts

OBJECTIVE: The thymic cysts are rare tumors of the neck and anterior mediastinum. The management of these patients in our institution is reported. Minimally invasive procedures are discussed. PATIENTS AND METHODS: Six patients operated in our institution within ten years, with a follow-up of 7.1 +/-3.7 years are studied retrospectively. RESULTS: There were four women and two men with an average of 39.8 +/-16.5 years. The tumor was found on chest radiograph in four asymptomatic patients, one took medical advice for laryngeal discomfort and another for dysphagia and dyspnea. The tumor was localized in the anterior mediastinum in three cases, in the cervicomediastinal site in two cases and in the cervical site in one case. CT scan was practiced in three patients with a mediastinal tumor and MR imaging in one of them. In patients with cervical or cervicomediastinal tumor, a cervical echography was practiced. All patients were operated on: three by cervicotomy, one by sternotomy, one by partial upper mini-sternotomy and one by right lateral video-assisted mini-thoracotomy. Histology confirmed benign epithelial thymic cyst. CONCLUSION: There is no specific marker of thymic cysts. Only the surgical management, leads to precise the diagnosis and to treat these tumors. No mortality, no complications or recurrences are reported. The minimally invasive surgery takes an interesting place for thoracic location, to explore and treat these benign mediastinal lesions.     

Hemifacial spasm due to tumor, aneurysm, or arteriovenous malformation.

The authors report eight cases of so-called symptomatic hemifacial spasm. They had gross pathological lesions such as a tumor (one epidermoid, one neurinoma, and two meningiomas), vascular malformation (one medullary venous malformation and two arteriovenous malformations), and aneurysm. In all four cases with a tumor, no artery compressed the facial nerve at the root exit zone. In three of the four cases, the hemifacial spasm disappeared after removal of the tumor in contact with the facial nerve. Compression or encasement of the facial nerve by the tumor was the pathogenesis of the hemifacial spasm in these three cases. The remaining case with tumor (tentorial meningioma) did not have a mass or vessel that directly compressed the facial nerve at the root exit zone. However, the hemifacial spasm disappeared after the removal of the tumor. In a case with a medullary venous malformation with arterial component, an engorged draining vein compressed the root exit zone of the facial nerve. In the remaining three vascular cases--two cases of arteriovenous malformation and a case of saccular aneurysm--enlarged feeding arteries and an aneurysm directly compressed the root exit zone of the facial nerve. Not only arterial or venous but also mass compression can cause hemifacial spasm in some symptomatic cases. Surgical decompression of the facial nerve from the causative organic lesion is the primary choice of treatment.     

Hematoporphyrin derivative and photoradiation therapy in early stage lung cancer

Photoradiation therapy (PRT) with administration of hematoporphyrin derivative (HPD) was performed in ten cases of early stage central-type lung cancer of which six cases were treated by PRT alone due to inoperability because of poor pulmonary function in five and refusal of surgery in one. Four cases were resected after PRT. In the former, complete tumor remission was obtained in all six and in the latter, complete tumor remission was seen in one and significant remission in three. In the nonresected cases four cases are disease-free at 17-41 months and the remaining two cases died of chronic obstructive lung disease and cerebral infarction 16 and 31 months after PRT, respectively. The resected cases are disease-free 7-32 months after surgery. Indications of PRT in early stage lung cancer are discussed according to histological findings of resected specimen.     

Retroperitoneal neurilemoma

Large retroperitoneal neurilemomas are rare. Five such cases are reported in four men and one woman varying in age from twenty-seven to seventy-five years. The tumor was found incidentally during examinations for other conditions in three cases. The other cases presented with abdominal pain, discomfort and increased abdominal girth. The tumors were all attached to the anterior surface of the sacrum. They varied in size from 12 cm in diameter to one containing more than 81 of fluid.     

Benign mucus-secreting tumors of the appendix. Six cases]

The authors report six cases of mucus-secreting tumor of the vermicular appendix, in four women and two men (mean age 59 years). All were benign. Pain in the right lower quadrant was the initial sign in 4 cases, while the mucus-secreting tumor was an incidental finding in the other two cases. A mass was palpated in the right lower quadrant in one case. A barium enema was performed in four cases and showed an extrinsic compression of the caecal base in one case, and of the right colon in another case. Four appendectomies, one associated with removal of the adjacent caecal tissues and two right colectomies were performed. Analysis of these six cases and a review of the literature allow us to recall the histologic classification of these tumors, which determines the prognosis and to emphasize the sonographic and CT scan findings which might lead to earlier preoperative diagnosis in the future. Management depends upon the circumstances under which the tumor is discovered, the local anatomy, and the type of tumor. Appendectomy is the treatment most often performed. Faced with a tumor of the appendix, without histologic proof or when the local anatomic conditions increase the risk of opening an abscess, right colectomy is recommended.     

Unusual presentations of cerebral solitary fibrous tumors: report of four cases

OBJECTIVE AND IMPORTANCE: Four rare cases of central nervous system solitary fibrous tumors (C-SoFTs) are described. This tumor has not previously been reported in children or in deep cortical structures. Three of these tumors occurred in the posterior fossa. Only four cases in the posterior fossa have been described previously. Nine years after its debulking from the posterior fossa, one tumor disseminated to the spine, lung, and liver. Only one such aggressive C-SoFT has been described previously. CLINICAL PRESENTATION: A 7-year-old child had had 1 year of right-sided weakness at presentation. Magnetic resonance imaging scans revealed a left basal ganglia lesion. A 49-year-old woman and a 30-year-old man presented after experiencing headaches for months. Magnetic resonance imaging scans in these patients revealed a tumor in the fourth ventricle and right jugular foramen, respectively. A 55-year-old man had spinal, liver, and lung dissemination of a previous posterior fossa tumor at presentation. INTERVENTION AND TECHNIQUE: All four patients underwent craniotomy for resection or subtotal removal of the tumor. Intraoperative observations noted solid well-encapsulated tumors. Immunohistochemistry confirmed C-SoFTs in all four cases. CONCLUSION: C-SoFTs are rare central nervous system, typically dural-based, tumors. They frequently are overlooked in the differential diagnosis of solid central nervous system tumors. Our findings suggest that these tumors can occur at any age and in most locations, regardless of proximity to the meninges (basal ganglia and ventricle), suggesting that the cells of origin are not meningothelial, but rather the mesenchyme of the cerebral vasculature. T1- and T2-weighted magnetic resonance imaging was notable for areas of hypointensity and of hyperintensity best described as patchy or a ying-yang appearance in all cases. Delayed extracranial metastasis may be noted.     

Bone tumors in the pelvis presenting growth during pregnancy

Among 56 cases of a giant cell tumor of bone (GCT) and 52 cases of chondrosarcoma (CSA) in our series, four patients were discovered to have a tumor in the pelvic bone that grew in size during pregnancy. These four rare cases are described here. They include three cases of a GCT in the sacrum and one case of a CSA in the innominate bone. The dextran-coated charcoal assay and immunohistochemical techniques demonstrated the independence of these tumors from hormonal regulation despite the growth stimulated during pregnancy. It was concluded that the delay in detection of these tumors in the pelvis was just related to the opportunity afforded for unexpected growth during pregnancy. Surgical management was difficult due to the delay in tumor detection. The initial complaints such as pain, discomfort, or numbness around the pelvis were misinterpreted as symptoms of pregnancy. It should be kept in mind that during pregnancy, any pain or numbness in the pelvic region could be the direct result of a tumor in the pelvic bone.     

Surgical treatment of giant cell tumors of the pelvis

There are many problems concerning management of giant cell tumors in the pelvis: diagnosis, surgical approach, adequacy of treatment, reconstruction, and prognosis. Of 58 cases of giant cell tumor of bone from 1962 to 1985, five cases arose in the pelvis, two in the ilium, two in the ischium, and one in the pubis. Surgical procedures included en bloc excision in four cases and intralesional excision in one. Reconstruction included sacroiliac fusion in one, iliofemoral fusion in one, and pelvic prosthetic replacement in another. Recurrence and metastasis did not occur in any of the cases. All patients were able to walk without crutches. A good surgical approach isolates major vessels and nerves, and permits excision to prevent recurrence and control bleeding. Reconstructive surgery may be required for treatment of a pelvic giant cell tumor.     

Diagnosis of the upper urothelial tumor using a rigid ureteroscope

We preoperatively diagnosed four cases suspected as malignant urothelial tumor of upper urinary tract using a rigid ureteroscope. In three cases of ureter tumor, biopsy was successful and showed low grade malignancy. Nephroureterectomy was performed in two cases with upper ureteral tumor. The tumor was fulgurated ureteroscopically in a case of lower ureteral tumor. In one case of renal pelvic tumor, biopsy was unsuccessful though the tumor was observed as a papillary configuration. Successively, biopsy using a flexible ureteroscope was performed and the tumor was treated by fulguration. Ureteroscopy is very useful for preoperative diagnosis of ureteral tumor and selection of treatment.     

Spindle cell neoplasms of lymph nodes of probable reticulum cell lineage. True reticulum cell sarcoma?

Primary spindle cell neoplasms involving hematolymphoid organs are extremely rare. We present four cases of spindle cell neoplasms of unusual phenotype arising within lymph nodes. Two of the four cases showed morphologic and immunophenotypic features suggestive of interdigitating reticulum cell lineage; these cases expressed several macrophage antigens and S-100 protein but not CD1. The other two cases showed evidence suggestive of dendritic reticulum cell lineage. Both cases expressed HLA-DR, several macrophage antigens, complement receptors C3b and C3d; one case expressed R4/23; both showed the presence of desmosomes on ultrastructural examination. A germline configuration for the immunoglobulin heavy chain and beta-T--cell receptor genes was detected in all four cases. Of the two patients in the first group, one had local recurrence of tumor; the other died of widespread metastases. Of the two patients in the second group, both are alive and well at 12 and 27 months follow-up, respectively.     

Acinic cell carcinoma of the lung ("Fechner tumor"). A clinicopathologic, immunohistochemical, and ultrastructural study of five cases.

The clinical, pathological, immunohistochemical, and ultrastructural features in five cases of primary acinic cell carcinoma of the lung are presented. The patients' ages ranged from 44 to 75 years (mean, 56); four were women and one a man. The lesions were discovered incidentally on routine chest x-ray and ranged from 1.2 to 4 cm in greatest diameter. Three tumors were located in the right middle lobe, one in the right upper lobe, and one in the left upper lobe. In three cases, the lesions presented as asymptomatic subpleural nodules in proximity to secondary bronchi, one case presented as an endobronchial tumor that led to obstructive symptoms, and one case as a well-circumscribed deep parenchymal nodule. Histologically, the tumors were composed of clear cells with abundant granular cytoplasm growing as solid sheets with focal acinar, microcystic, and papillocystic areas. Immunohistochemical stains showed strong positivity of the tumor cells for low-molecular-weight cytokeratins and epithelial membrane antigen (EMA). Focal weak cytoplasmic positivity was observed in three cases with alpha-1-antichymotrypsin and in one case with amylase. Stains for vimentin, S-100 protein, chromogranin, and lysozyme were negative in all cases examined. Electron microscopy performed in four cases showed abundant zymogen-type cytoplasmic granules of variable electron density characteristic of acinar-type secretory cells. All patients were treated by lobectomy alone. Follow-up of 3 to 10 years in four cases revealed that all patients were alive and well, with no evidence of recurrence or metastases. Because of their relatively indolent behavior and favorable prognosis, primary acinic cell carcinoma of the lung must be distinguished from other primary and metastatic clear cell tumors of the lung.     

Stewart-Treves syndrome

Lymphangiosarcoma is an uncommon vascular tumor that usually develops in longstanding lymphedema. We gathered the cases of lymphangioma observed in a hospital and attempted to analyze their characteristics: age, sex, localization, treatment and follow-up data. We studied five cases: three cases of Stewart-Treves syndrome after mastectomy and radiotherapy and two cases that developed in patients with late-onset congenital lymphedema. There were four women and one man. Radical surgery was performed in four patients. The techniques employed were: above-knee amputation (one patient), hip disarticulation (one patient), scapulo-humeral disarticulation (two patients) and gemcitabine and radiotherapy in one patient with metastatic disease at diagnosis. Three patients died in the first 14 months of follow-up, while two are disease free after 46 and 86 months respectively. This study confirms the poor prognosis of patients with Steward-Treves syndrome.