乳腺癌相关多原发癌

目的 探讨乳腺多原发癌诊断、治疗及预后。方法 回顾性分析乳腺癌相关多原发癌6例资料。第一、二原发乳腺癌均经根治性手术或加故疗及化疗。结果 随访其中3例(50％)有转移性复发。结论 免疫功能抑制和肿瘤生物学行为，可能是乳腺原发癌复发及第二原发癌发生的原因之一。     

Pilomatrix carcinoma

Summary Malignant tumours of hair matrix origin are unusual and have only recently been widely recognised. We herein report one such case and review the current literature.     

Adrenocortical carcinoma

In reviewing the experience of a number of authors and investigators, it is clear that early diagnosis of adrenocortical carcinoma is essential for cure. Of all the modalities of therapy currently available, surgical resection holds the most promise for cure or prolonged survival. Treatment for extensive local disease or metastatic disease has been discouraging, and the prognosis for reasonable, comfortable survival is poor. Unfortunately, the toxicity of mitotane, an adrenolytic agent and currently the most effective drug available, is often unacceptable and may militate against its use. Because many of the debilitating side effects of these tumors are related to hormone production, newer drugs that result in hormonal blockade may add considerably to the comfort of the patient. The development of less toxic chemotherapeutic agents presents a challenge for both the oncologist and the endocrinologist.     

Cortico-suprarenal carcinoma

The authors show the case of a 69 years old male with a large corticosuprarenalian tumor that was detected on an random abdominal echographic examination. The patient was operated in the General Surgery Department. of Prof. Agrippa Ionescu Hospital, Bucharest. We performed ablation of the large left suprarenalian gland malign tumor with left nephrectomy, splenectomy and partial pancreatectomy. The hystopathological examination reveals a diffuse corticosuprenalian carcinoma. The case is interesting because of low incidence of this kind of malign tumor and also of the unusual tumor evolution in a long time up to its large size (12 cm in diameter).     

Appendiceal carcinoma masquerading as primary bladder carcinoma.

We report 2 cases of appendiceal adenocarcinoma invading the bladder. Both tumors masqueraded as primary bladder carcinomas. Cystoscopic biopsies obtained adenocarcinomatous tissue and the secondary nature of the bladder lesion became apparent at laparotomy in both cases. One patient was treated with surgical resection of the appendix, the adjacent cecal wall and the bladder wall, and postoperative irradiation. She was well 10 years later. The other patient was treated with right colectomy and segmental bladder resection. She died of diffuse peritoneal recurrence 6 years later.     

Invasive carcinoma of prostate presenting as rectal carcinoma

Prostate carcinoma occasionally can present with rectal obstructive symptoms and an annular constricting lesion of the rectum. Discriminating between primary rectal carcinoma and prostate carcinoma locally invasive to the rectum is of obvious importance because of the different treatments and prognoses. History and physical examination play only a marginal role in differentiating between these two lesions. The diagnosis of prostatic malignancy in patients in this circumstance can be supported by an elevated serum acid phosphatase as well as a bone scan that demonstrates a pelvic/vertebral distribution of bony metastases. The rectal mucosa is usually spared, and a barium enema often will demonstrate tapered margins as opposed to a tumor edge in primary rectal malignancy. Excretory urography often demonstrates hydronephrosis. Rectal biopsy with immunohistochemical staining for prostate specific antigen can direct the origin of a poorly differentiated adenocarcinoma to the prostate. Treatment involves hormonal manipulation with estrogen therapy or orchiectomy. Radiation therapy to the obstructed rectum has provided satisfactory palliation when hormonal manipulation fails.     

Urothelial carcinoma

Various study groups are working on the WHO classification of 2004 which eliminates the previous grades of differentiation G1, G2, and G3 and classifies non-muscle-invasive bladder cancer into genetically stable low-grade and genetically unstable high-grade urothelial carcinomas. In muscle-invasive bladder cancer, extended lymph node dissection as part of radical cystectomy should remain the standard procedure for now.     

Adrenocortical carcinoma

Ten patients, seven women and three men, ages 47 to 76 years (mean 58.6 years), treated for adrenal cortical carcinoma between 1971 and 1989, were reviewed. Three (30%) of the tumors were nonfunctioning. The remaining seven (70%) were functioning, six of them occurring in women. Common presenting features were hormonal excess, distant metastases, weight loss, and abdominal pain. The primary tumor was resected in all patients, only two of whom had disease confined to the adrenal gland. Tumor diameter ranged from 9 to 21 cm (mean 15.7 cm). Inferior vena caval or right atrial extension of tumor thrombus was present in two patients. Excluding two deaths from postoperative complications, seven patients died of their disease after a mean survival of 25 months (range 2 to 84 months). Of seven patients who received o,p'-DDD treatment for metastatic or recurrent tumor, three (43%) had an objective response. In two patients, tumor regression was complete and was associated with prolonged survival. The first patient underwent resection of recurrent tumor on two occasions in addition to receiving o,p'-DDD and survived 84 months. The second patient had complete regression of pulmonary and liver metastases confirmed at laparotomy and thoracotomy and remains free of disease at 78 months. None of the five patients treated with various combinations of cytotoxic chemotherapy had an observable response, and no measurable effect was seen in a single patient following abdominal radiotherapy. It is concluded that resection for local recurrence may prolong survival and that significant and lasting tumor regression is possible with o,p'-DDD administration. Beneficial results from cytotoxic agents, however, could not be demonstrated.     

Bronchial carcinoma

From a retrospective review of 4,000 patients with bronchial carcinoma, managed in the Thoracic Surgical Unit in Edinburgh, the salient features of presentation, surgical management, and the natural history in those unsuitable for surgical management are reviewed.     

Parathyroid carcinoma

BACKGROUND: Parathyroid carcinoma is a rare malignancy affecting 0.5-5 per cent of all patients with primary hyperparathyroidism. This article reviews the literature on the pathogenesis, pathology, clinical features, diagnosis and management of parathyroid carcinoma. METHODS: A Medline search was performed and all relevant English language articles published between 1970 and 2005 were retrieved. The search words included 'parathyroid carcinoma', 'pathology', 'genetics', 'management' and 'radiotherapy'. Secondary references were obtained from key articles. RESULTS AND CONCLUSION: The exact aetiology of parathyroid carcinoma remains obscure. Recently, the HRPT2 gene has been implicated in its pathogenesis and may prove to be a genetic target in future. Surgical resection is the accepted 'gold standard'. There is now a growing consensus on the use of adjuvant radiotherapy as it has been shown to provide a survival benefit.     

Adrenocortical carcinoma

Adrenocortical carcinoma is rare, tends to occur in the first decade as well as the fourth and fifth decades of life, and is slightly more common in women. The tumors are classified as functional or nonfunctional, depending on tumor production of corticosteroid, androgen, estrogen, or mineralocorticoid. Most patients present with large masses and with stage IV disease. Abdominal computerized tomography and magnetic resonance imaging are used in the evaluation of intra-abdominal disease. The most effective treatment for adrenocortical carcinoma is complete resection. Surgical resection remains the only potentially curative treatment for this disease. Early stage and curative resection are the two clinical factors that are of prognostic significance for long-term survival. Mitotane is the chemotherapeutic agent most often used to treat adrenocortical carcinoma. Its efficacy in prolonging survival is limited but may be enhanced by monitoring of serum levels and their maintenance at elevated values. Even for patients who undergo complete resection, recurrent and metastatic disease are extremely common. The only effective treatment for recurrent disease is reoperation.     

Urachal carcinoma

The clinical and morphologic features in 14 cases of urachal adenocarcinoma are reviewed. The disease occurred more frequently in men than in women. Seven patients (50%) remain alive and currently free of disease at periods ranging from fifteen months to ten years (median: 6 years). Neither the histologic appearance nor the differentiation of the tumor appeared to affect prognosis. It is concluded that the criteria previously recommended to establish the diagnosis of this rare malignancy were too restrictive.