Primary chronic sclerosing osteomyelitis--a case-report

A case of primary chronic sclerosing osteomyelitis of the fibula in a 14-year-old is described. This rare condition can be difficult to differentiate from a bone tumor. Its pathogenesis is controversial, although the prevalent hypothesis involves chronic osteomyelitis developing after an unrecognized phase of acute infection. The existence of primary chronic sclerosing osteomyelitis as a disease in its own right has been challenged. Recently, primary chronic sclerosing osteomyelitis has been interpreted as a localized or monofocal variant of multifocal recurrent chronic osteitis or of the bone abnormalities associated with seronegative spondyloarthropathies (SAPHO syndrome).     

Bone metastases from bronchial carcinoid tumors. Two case-reports

Two case-reports of metastatic bone disease in patients with bronchial carcinoid tumors illustrate the diagnostic challenges raised by these slowly-growing malignancies of which the primary frequently escapes early identification. The first patient had the typical picture of a primary with a single bone metastasis. Unusual features in the second patient were the large number of bone metastases, involvement of distal skeletal sites, and elevation of serotonin and 5-hydroxyindoleacetic acid levels.     

Gastrointestinal Carcinoid Tumors: Factors that Predict Outcome

Gastrointestinal (GI) carcinoids are neuroendocrine tumors originating in multiple locations throughout the GI tract. The prognosis for patients with GI carcinoid tumors is diverse. To determine the factors that significantly affect prognosis, we reviewed our experience. Between 1992 and 2000 a total of 70 patients with GI carcinoid tumors underwent surgical resection at our institution. The patients were grouped into three categories based on the origin of the carcinoid tumor: foregut, midgut, hindgut. The mean age of the patients was 56 ± 2 years. All patients with foregut carcinoids had symptoms upon presentation, whereas 61% of those with midgut carcinoids and only 37% of those with hindgut carcinoids had symptoms (p < 0.001). The factors that most strongly affected survival on univariate analysis were a symptomatic presentation and the site of origin. Patients with foregut or midgut lesions had lower 5-year disease-free survivals than those with hindgut tumors. Moreover, the size of the primary tumor and the presence of liver metastases were not independent predictors of survival. Despite the larger tumor size and the higher incidence of liver metastases, patients with foregut carcinoids appear to have the same prognosis as those with midgut carcinoids. These data therefore suggest that the outcomes of patients with carcinoid tumors are highly dependent on the presence of symptoms and the site of origin.     

Primary chronic sclerosing (Garré's) osteomyelitis in children.

Three children with unifocal nonpyogenic inflammatory bony lesions with a prolonged, fluctuating course are reported. The lesions were located at the metaphyseal region of long bones. Three was progressive sclerosis and hyperostosis in the tibia or femur, such as the changes described in Garré's osteomyelitis. No pus was released by exploration of the lesions. Tissue and blood cultures were negative. The histology was typical of chronic osteomyelitis: the symptoms returned intermittently over several years, together with the development of sclerosis but without disturbance of bone growth. It is not clear whether Garré's chronic sclerosing osteomyelitis is a different entity from chronic recurrent multifocal osteomyelitis.     

A case of cervical metastases from temporal bone carcinoid

BACKGROUND: Carcinoids are neuroendocrine tumors that typically occur in the gastrointestinal tract and lung and less frequently in the head and neck region. Whereas the metastatic potential of laryngeal carcinoid is well documented, only one case of metastasis of temporal bone carcinoid to the cervical lymph nodes has been reported. We present the case of a 55-year-old woman with cervical metastases almost 3 years after a primary diagnosis of temporal bone carcinoid. METHODS: The patient had undergone a subtotal petrosectomy for a temporal bone carcinoid and, 29 months later, revision surgery for a local recurrence. Four months later, multiple laterocervical swelling appeared, and the patient underwent modified radical neck dissection. RESULTS: Histologic examination after the neck dissection was suggestive of lymph node metastases from a carcinoid tumor. Immunohistochemical analysis of the lymph node metastases showed neoplastic cells positive for keratin, vimentin, chromogranin, and neuron-specific enolase and negative for thyroglobulin, calcitonin, S-100, and parathyroid hormone. The patient was treated with intensity-modulated radiation therapy and neck irradiation. CONCLUSIONS: We believe that temporal bone carcinoids have metastatic potential not predictable by histologic features. Surgical excision is the treatment of choice for patients with temporal bone carcinoids, with the approach and technique determined by the extent of the mass. Moreover, before planning surgery and during follow-up, neck node status must be carefully detected.     

Gastrointestinal carcinoids: characterization by site of origin and hormone production

OBJECTIVE: To describe a large series of patients with carcinoid tumors in terms of presenting symptoms, hormonal data, stage at diagnosis, pathologic features, and survival. SUMMARY BACKGROUND DATA: Published series have described significant prognostic features of carcinoid tumors as site of origin, age, sex, stage at diagnosis, presence of high hormone levels, and increased T stage. Of these, stage at diagnosis and T stage seem to emerge most often as independent predictors of survival in multivariate analyses. Of carcinoid tumors, those arising from a midgut location have higher levels of serotonin and serotonin breakdown products, as well as more frequent metastatic disease at presentation, than those arising from either foregut or hindgut locations. METHODS: A prospective database of carcinoid patients seen at Duke University Medical Center was kept from 1970 to the present. Retrospective medical record review was performed on this database to record presenting symptoms, hormonal data, pathologic features, and survival. Statistical methods included analysis of variance, Kaplan-Meier analysis, and Mantel-Cox proportional hazard survival analysis, with P <.05 considered significant for all tests. RESULTS: Carcinoids arising in different locations had different presentations: rectal carcinoids presented significantly more often with gastrointestinal bleeding, and midgut carcinoids presented significantly more often with flushing, diarrhea, and the carcinoid syndrome. Patients with midgut tumors had significantly higher levels of serotonin and serotonin breakdown products, corresponding to higher metastatic tumor burdens. Although age, stage, region of origin, and urinary level of 5-hydroxyindoleacetic acid predicted survival by univariate analysis, only the latter three were independent predictors of survival by multivariate analysis. Of the patients with metastatic disease at diagnosis, those with midgut tumors had better survival than those with foregut or hindgut tumors. CONCLUSIONS: Although region of origin is certainly an important factor in determination of prognosis, stage of disease at presentation is more predictive of survival. Pancreatic and midgut carcinoids are metastatic at diagnosis more often than those arising in other locations, leading to a worse overall prognosis. Among patients with distant metastases, patients with midgut primary tumors have improved survival despite increased hormone production compared with patients with tumors arising in other primary sites.     

Nonsurgical Treatment of Advanced Malignant Neuroendocrine Pancreatic Tumors and Midgut Carcinoids

Neuroendocrine enteropancreatic tumors are relatively uncommon and present variable biologic and clinical features. Surgery at early disease stages is currently the only available cure. Evidenced-based medicine and consensus on systemic treatment of advanced malignant disease is virtually nonexistent, and the various available therapeutic regimens should be considered primarily palliative. Symptomatic treatment of patients with endocrine functioning tumors, however, is of utmost importance and leads to extensive prolongation of survival. Biotherapy by means of somatostatin analogs and interferon-α facilitates symptomatic control and offers stabilization of tumor progression for extended periods of time. Pancreatic endocrine tumors metastatic to the liver frequently respond to chemotherapy by means of a significant reduction in tumor volume. Programs including streptozotocin and 5-fluorouracil or doxorubicin are generally regarded as first line treatment for pancreatic endocrine tumors with liver metastases, whereas midgut carcinoid tumors often are resistant to systemic chemotherapy. Treatment attempts in patients with the latter disease may rather include interferon-α and somatostatin analogs. Somatostatin receptor-targeted radiotherapy is still investigational. Repeated surgical intervention including various debulking procedures should be considered here and in patients with advanced neuroendocrine pancreatic tumors and midgut carcinoids requiring systemic antitumor treatment.     

Evaluation, prognosis, and medical treatment considerations of metastatic bone tumors.

Metastatic cancer can cause severe pain and disability. Metastases can occur in any bone, but usually are located in the axial or proximal appendicular skeleton. The most frequently encountered primary tumors that spread to bone are those of the prostate, breast, kidney, lung, and thyroid. When the origin of the primary cancer is known, skeletal metastases are more often from breast or prostate. When the primary site is unknown, the lung and kidney should be suspected as sites of origin. The nonoperative management of skeletal metastases from multiple myeloma and from carcinomas of the prostate, breast, kidney, lung, and thyroid are discussed.     

Surgery and Radiofrequency Ablation for Treatment of Liver Metastases from Midgut and Foregut Carcinoids and Endocrine Pancreatic Tumors

Background Many neuroendocrine tumors (NETs) have a tendency to metastasize to the liver. In case of limited number of metastases, liver surgery or radiofrequency ablation (RFA) may result in apparently total clearance of metastases. However, it is not clear whether such therapy will provide symptom reduction or increased survival. Methods Seventy-three patients with foregut (n = 6) or midgut carcinoids (n = 37) or endocrine pancreatic tumors (n = 28), and two patients with NETs without discernable origin were studied. Symptoms were evaluated using a Symptom Severity Score. Liver surgery was performed in 42 operations and RFA on 205 lesions. Results Apparently total clearance of liver metastases was attained in 1 of 6 patients with foregut carcinoids, 15 of 37 with midgut carcinoids, and 13 of 28 with EPT. Symptom improvement was noted in 12 of 17 (70.6%) patients with carcinoid syndrome, and 75% also reduced their 5-HIAA and P-CgA by at least 50%. Patients with nonfunctioning EPT generally had no improvement of symptoms after surgical/RFA liver treatment, but eight patients had functioning EPT, and four of these reduced their biochemical markers by at least 50%. NETs with higher Ki67 index tended to recur more often. Complications occurred in 9 of 45 open surgery procedures, and in 8 of 203 RFA procedures. Conclusions Treatment of liver metastases is successful in midgut carcinoid patients with limited liver metastases. Patients with foregut carcinoid and EPTs recur more often, possibly related to higher Ki67 index, and treatment of liver lesions less often reduces symptoms. Liver resections and RFA may be safely performed, and RFA is associated with few complications.     

Method for Dissection of Mesenteric Metastases in Mid-gut Carcinoid Tumors

With adequate medical management the midgut carcinoid tumor generally is an indolent malignancy associated with substantial life expectancy and appreciable life quality, even in the presence of liver metastases and significant tumor burden. Abdominal complications may occur in this entity of carcinoids owing to entrapment of intestines and encasement of mesenteric vessels by mesenteric metastases and associated marked mesenteric fibrosis. This may be the cause of abdominal pain, disabling diarrhea, weight loss to the extent of malnutrition, and eventually the risk of death with acute or chronic intestinal obstruction or intestinal gangrene. Operative removal of the mesentericointestinal lesion is often indicated to prevent or treat these complications but may be technically difficult when mesenteric metastases extend in the vicinity of major vessels in the mesenteric root. At laparotomy 56 patients with advanced midgut carcinoids underwent removal of the mesenteric tumor with a method for preserving the mesenteric vessels. This was feasible by mobilizing and releasing the right colon and mesenteric root from posterior adhesions, identifying the mesenteric artery below the pancreas, and free-dissecting this artery on the tumor capsule in the mobilized mesentery. Dissection was successful even with tumors initially judged inoperable unless tumor growth completely surrounded the mesenteric vessels or extended retroperitoneally. One patient was subjected to distal intestinal artery bypass. Symptom relief was been substantial and often of long duration after mesenteric tumor removal in patients who prior to surgery often had threatening intestinal ischemia. Patients with advanced midgut carcinoids may benefit markedly from dissectional removal of mesenteric tumors, which (conceivably better than conventional wedge resection) preserves the length of the remaining intestine.     

Surgical treatment as a principle in patients with advanced abdominal carcinoid tumors.

Seventy-five patients with advanced abdominal carcinoid tumors (65 midgut, 10 others) have been examined retrospectively to evaluate the role of surgical treatment as a principle, irrespective of stage of disease. Eighteen of 52 patients (35%) exhibited the carcinoid syndrome. Two or more primaries were found in 39% of patients with midgut lesion, 81% of these patients had regional metastases, 5% of these patients had distant lymph node metastases, and 74% of the patients had liver secondaries. All patients underwent operation, an additional 34% of the patients had a further reoperation, 9% of the patients had a second reoperation, 3% of the patients had a third reoperation, and one patient (2%) had a fourth reoperation. Intraoperative debulking (liver excluded) was performed in 33% of the patients, and 48% of the patients had treatment (resection, hepatic artery ligation, embolization) directed at the liver. The postoperative mortality rate was 2% after the primary operation for midgut lesions. The median survival for midgut tumors was 92 months, compared to 40 months for other lesions (not significant). A significantly higher survival rate was revealed for those patients with midgut lesion who were undergoing intraabdominal debulking procedures (liver excluded); median survival was 139 months versus 69 months without debulking. For those patients with liver metastases, median survival after intervention was 216 months and 48 months without such treatment (p less than 0.001). It is concluded that resection of intraabdominal carcinoid tumor masses can be performed in a high proportion of patients. Despite the retrospective, uncontrolled nature of this study, the difference in survival probabilities in favor of aggressive surgical therapy is so marked that it is not unreasonable to conclude that surgery has played a role in prolonging life in these patients.     

Carcinoid tumors in the gastrointestinal tract--an analysis of 156 cases

A series of 156 patients with gastrointestinal carcinoid tumors is presented. The tumors were grouped as foregut, midgut and hindgut carcinoids. The appendiceal tumors were grouped separately. Lack of specific symptoms resulted in only 26% correct presumptive diagnoses. This also reflected the patient's delay greater than 2 years in 10% and doctor's delay greater than 2 years in 29%. In the larger group (midgut), 41% were multiple primary tumors. Liver metastases were found in 46% and tumors bigger than 1 cm metastasized to the liver in 58%. The five-year survival of patients with liver metastases was 43% while the figure for those without metastases was 80%. In 47 patients with appendiceal carcinoids, 68% had tumors less than 1 cm. No metastases to the regional lymph nodes, viscera or skeleton were found. Thirty-three patients were traced and none had died of the tumor. Two foregut and 4 hindgut tumors were re-examined using immunocytochemistry. In one of the foregut tumors, cells containing serotonin were found while the other one contained gastrin cells. In 3 hindgut tumors PP-cells were seen and one of them also harbored glucagon/glicentin cells. In one hindgut tumor no peptides were found.     

Chronic sclerosing osteomyelitis of Garré affecting fifth metatarsal bone of the foot

We describe a 20-year-old woman with a 10-year history of Garré’s chronic sclerosing osteomyelitis occurring in the fifth metatarsal bone of the left foot. Apart from the rarity, this case illustrates the difficulties in diagnosing and effectively treating this chronic non-suppurative bone infection.     

Chronic sclerosing osteomyelitis. Apropos of 4 cases and the value of the treatment by closed intramedullary reaming

Chronic sclerosing osteomyelitis is a rare variety of primary osteomyelitis located principally on the diaphysis of the long bones in adults. Pain is the main symptom, but biological signs are negative or normal, and histological examination is non-specific. X-rays are very stereotyped with enlargement of the cortex of diaphyseal bone. This disease is note worthy by its difficult diagnosis, and is often misdiagnosed as bone tumor, malignant or benign, especially osteoid osteoma. Four observations are reported and in one case, after 13 years, an abscess developed, bearing out the oneness of chronic, primary osteomyelitis. This form deserves its individualization because of its treatment by closed intra-medullary reaming which proves to be effective after an average follow-up of 10 years.     

Surgery as primary treatment in patients with liver metastases from carcinoid tumors: a retrospective, unicentric study over 13 years

BACKGROUND: The heterogeneous nature of carcinoid tumors makes it difficult to develop a standardized treatment strategy for the primary tumor itself and for probable liver metastases. However, prolongation of the 5-year survival rate (5-ysr) and amelioration of the incapacitating symptoms after resection of the primary tumor and its metastases demonstrate that surgical intervention must be the treatment of choice in these tumors. METHODS: The data of 31 patients (17 patients with midgut carcinoids, 10 patients with an endocrine carcinoma (carcinoid) of the pancreas, and 4 patients with carcinoids of the lung) who underwent liver operation for metastatic carcinoid tumors between 1983 and 1996 were analyzed, with special regard to factors influencing postoperative survival. RESULTS: Ten patients underwent curative resection (5-ysr, 86%), and palliative operations were performed in 21 patients (5-ysr, 26%). The overall 5-ysr was 47%, with a mean postoperative follow-up of 3.5 years (range, 4 months to 10.8 years). Postoperative morbidity rate was 13%. Size of liver metastases, radicality of the operation and localization of the primary tumor were factors influencing postoperative survival. CONCLUSIONS: Surgery for metastatic carcinoid tumors may be curative or palliative, with a potential for cure in some cases and prolongation of survival and amelioration of symptoms in the majority of patients.     

Progression of Metastases and Symptom Improvement from Laparotomy in Midgut Carcinoid Tumors

n = 59) generally was accompanied by symptoms of abdominal pain and weight loss, and it often required urgent intervention due to intestinal obstruction or ischemia. Complete or partial symptom alleviation was accomplished in 82% of the operated patients, and generally was most auspicious after primary acute or subacute procedures ( n = 54). The complete or partial symptom improvements after surgery lasted for mean 5.3 years and tended to be longer after elective ( n = 50) than acute operations. The findings substantiate encouraging results of laparotomy in a compromised cohort of patients with midgut carcinoid tumors. Because the patients also displayed a generally slow progression of metastases, liberal indications for laparotomy should prevail in symptomatic and possibly also asymptomatic individuals with midgut carcinoid tumors.     

Carcinoid tumors of the gut: Diagnosis and treatment

Carcinoids are relatively uncommon tumors, which may occur anywhere along the gastrointestinal tract. The appendiceal carcinoid is in autopsy series the most prevalent, but frequently overlooked clinically unless routinely searched for at appendectomy. In the stomach and the rectum the carcinoids often present as simple benign polypous lesions, fortuitously discovered during endoscopy, while the rare colon carcinoid is generally a large, bulky tumor at time of diagnosis. The mid-gut carcinoid is most frequently located in the terminal ileum and tends to prevail in clinical series due to its common association with the carcinoid syndrome. Ventricular, rectal and appendiceal carcinoids are generally benign and the extent of surgery may safely be based on the size of the tumor. Local excision or simple appendectomy is thus generally sufficient and only the larger, invasive or clearly malignant lesions require more extensive surgery. The mid-gut carcinoids on the other hand are frequently malignant, irrespective of tumor size, and surgery should carefully aim to remove mesenteric metastases together with the primary tumor. Also in cases with disseminated mid-gut carcinoids tumor debulking of the larger mesenteric and liver metastases may cause considerable alleviation of symptoms and probably facilitates medical treatment of the carcinoid syndrome. Surgery may also relieve abdominal symptoms due to intestinal entrapment or impaired intestinal circulation, which is frequently encountered in association with the more advanced mid-gut carcinoid.     

Oral complaints caused from metastases to the mandible and maxilla

Jaw bone disorders causing oral complaints are common in primary care settings. Most of these conditions are of a chronic and benign nature. However they also may be the symptoms of a primary or secondary malignant process in the bone. The most common malignant bone tumor is metastatic carcinoma, and tumors arising in the breast, prostate, thyroid, lung and kidney have a special propensity to spread to bone. Yet metastases to the bones are rare; less than one per cent of all neoplasms metastases to the maxillofacial area. We describe four cases of metastatic tumours to the jaws. Two cases originated from the thyroid gland while the rest were from the oesophagus and the liver respectively. Three lesions occurred in the mandible and one in the maxilla. Patients presented with oral discomforts disregarding the primary tumor. Physicians who frequently advise patients with oral complaints should keep in mind that whereas these symptoms are mostly of a chronic and benign nature, metastases from a malignant tumor must be included in the differential diagnosis.     

Carcinoid tumor of the breast: Treatment with breast conservation in three patients

Background: Carcinoid tumors of the breast have been described in the literature. The diagnosis is made by identification of typical histologic features and confirmed by a positive argyrophilic reaction or the presence of neurosecretory granules. There are several theories of the pathogenesis of carcinoid tumors in the breast and controversy as to whether these tumors actually originate in the breast ducts or are tumors that arise from neuroectodermal cells that have migrated to the breast ducts. Historically, treatment of carcinoid of the breast has been by mastectomy. Methods: We report three cases of primary carcinoid tumor of the breast treated with lumpectomy and axillary node dissection. No adjuvant radiation or systemic treatment was administered. Results: In all three cases, no metastases were identified in lymph nodes sampled and all patients have remained clinically free of recurrent disease. Conclusions: Decisions about the need for radiation or systemic treatment of breast carcinoid tumors depend on one's interpretation of the pathogenesis of this disease. Breast conservation is a surgical option that has not been previously reported. Larger series of carcinoid tumors of the breast, their treatment, and their follow-up are needed.     

原发性肾脏类癌2例报告并文献复习

目的：探讨原发性肾脏类癌的临床特征及诊治方法。方法：总结2例原发性肾脏类癌患者的临床资料结合文献复习讨论。例1,女,48岁。因右腰酸痛1个月入院。B超示右肾中上极中等回声占位,边界清楚,内有血流信号。CT示右肾中上极10．0cm×7．5cm实性占位,增强后病灶均匀轻度强化。例2,男,44岁。体检发现左肾占岱40天入院。B超示左肾门处中等回声占位,边界清楚,内有血流信号。CT示左肾中极肾门处3．7cm×3．5cm实性占位,增强后病灶均匀轻度强化;其内伴-钙化点。结果：2例均行根治性肾切除术。病理检查镜下肿瘤细胞形态为多边形,嗜酸性颗粒状细胞质,边界不清,呈柱状或缎带状排列混合有实性巢状排列;细胞核呈园形大小一致,核分裂像难见。免疫组化：突触素、波形蛋白均为阳性。病理诊断为肾脏类癌伴淋巴结转移。例1术后30个月死于肝骨转移;例2术后随访26个月,未见肿瘤复发转移。结论：原发性肾脏类癌临床罕见,易发生淋巴结转移,确诊依靠病理学检查,根治性肾切除应为治疗原发性肾类癌的首选方法。