Primary carcinoid tumor of the testis: case report, ultrastructure and review of the literature.

A case of primary pure carcinoid tumor of the testis which occurred in a 71-year-old male is reported. The patient was treated by radical orchiectomy and remains well and symptom free 10 months after operation. Histologically as well as ultrastructurally the tumor showed typical appearances of carcinoid tumor of midgut derivation. 23 cases of carcinoid tumors of the testis were discovered in the literature. Of these 17 were primary testicular carcinoids, and 6 were metastatic to the testis. Of the 17 cases of primary carcinoid tumors, 14 were pure carcinoids and only 3 were associated with teratoma. None of the primary testicular carcinoids were associated with metastases and the prognosis after orchiectomy was excellent, thus indicating that no further therapy is necessary. The prognosis of patients with carcinoid metastatic to the testis is poor. In view of this it is very important to determine whether the tumor is primary or metastatic.     

气管支气管类癌20例

目的 了解气管、支气管类癌的临床生物学特性,探讨合理的手术指征及术式。方法 对20例气管、支气管类癌患者的外科治疗结果进行综合分析。结果 气管、支气管炎癌的临床特点和一般肺癌相比缺乏特征性表现,免疫组化结果提示其为神经内分泌细胞来源。20例均予以手术切除,根治性切除率95．0％,手术并发症发生率10．0％。术后病理报告典型类癌13例,淋巴结转移率为23．1％,;不典型类癌7例,淋巴结转移率为3／7。13例典型类癌的5,10,15年生存率分别为100．0％、7／8及3／4。7例不典型类癌中,6例存活患者术后尚未满5年,1例术后2年余死于远处转移。结论 手术切除是治疗类癌的主要手段。手术指征不应限于Ⅲa期及分期较早患者,对可能切净的Ⅲb期患者也应尽早手术。术式的选择应主要考虑患者的年龄及肺功能状况,对年龄、肺功能正常者,应以手术根治性为目的,否则应选择保守术式。     

Synchronous primary cancers of trachea and esophagus and ventricular tachycardia

The incidence of multiple primary cancers involving trachea is rare. We present a case of synchronous double primary cancer of trachea and esophagus in a 70-year-old woman, with a special symptom of ventricular tachycardia and no/a/story of smoking and alcohol drinking. Biopsies （tom multiple loci demonstrated the patient had primary small cell cancer of trachea and squamous cell carcinoma in situ of esophagus. The patient was successfully treated with four cycles of chemotherapy consisting of etoposide and earboplatin （EC） followed by thoracic radiotherapy （60 Gy in 30 fractions, in 6 weeks）, and was evaluated to have complete response of tumor. To our knowledge, there is no synchronous cancer of trachea and esophagus has been reported in English literature, and our experience showed sequential EC chemotherapy and radiotherapy provided an effective treatment to control both cancers.     

Tracheal carcinoma presenting as a thyroid tumor

Direct invasion of the trachea by an aggressive tumor originating in the thyroid gland is an unusual but well-documented phenomenon. The reverse situation, that is, a primary tracheal neoplasm invading by direct extension into the thyroid gland and presenting as a thyroid mass is apparently an even less common occurrence. A careful search of the literature has failed to reveal any recorded instances of this type of occurrence. We report a case of an adenoid cystic carcinoma arising in the trachea and invading the thyroid gland and recurrent laryngeal nerves causing vocal cord paralysis. The presentation of this lesion as a primary thyroid tumor is an unusual occurrence that serves to remind the clinician that in the differential diagnosis of thyroid masses one must include the possibilities of metastases or direct extension of a primary neoplasm from another organ.     

The use of Alu-PCR to distinguish between typical pulmonary carcinoids versus classic midgut carcinoids

Although histologically 'typical' pulmonary and 'classic' midgut carcinoids are similar, the small intestine tumors are more aggressive than their pulmonary counterpart. We believe that in contrast to pulmonary carcinoids arising from Kulchitsky cells, 'classical' midgut carcinoids develop from crypt stem cells that differentiate into endocrine ('classical') or exocrine-endocrine ('non-classical' adenocarcinoid) tumors. The different progenitor cells may determine different malignant potentials between these types of carcinoids. To identify genetic differences between 'classical' midgut and typical pulmonary carcinoids using an Alu-PCR genomic profiling method, we reviewed 54 cases of carcinoid tumors that were surgically removed at Hartford Hospital from 1996 through 2001. Histologically these cases were selected into three groups: i) foregut or pulmonary carcinoids, ii) 'classic' midgut carcinoids of small intestine and iii) multiple typical pulmonary carcinoids. Genomic-profiling of DNA from these cases was performed using an Alu-PCR method. Metastases were observed in 18/20 'classical' intestinal carcinoid tumors, 3/30 pulmonary carcinoids, and 0/4 multiple pulmonary carcinoids. These results confirm that pulmonary carcinoids behave in a more benign fashion than intestinal carcinoid tumors. Using Alu-PCR to profile tumor cell genomic DNA, we showed that 68% of small intestine carcinoids and 58% of pulmonary carcinoids had allelic banding patterns suggestive of either amplification or deletion of gene sequences. Alu-PCR demonstrated loss or gain of genetic sequences that were unique for each examined group. These findings strongly suggest that pulmonary carcinoids differ from their intestinal counterparts.     

Primary Renal Malignant Fibrous Histiocytoma Four-Case Report and Review of the Literature

OBJECTIVE To evaluate the diagnosis and treatment for primary renal malignant fibrous histiocytoma, a rare tumor arising from the kidney.METHODS The clinical and pathological data from 4 cases of malignant fibrous histiocytoma of the kidney detected in our hospital are described.One case of special interest involved a giant cell subtype, the first to be reported in the oncology literature. The clinicopathologic features and prognostic factors of this tumor were analysed and summarized after reviewing 55 documented cases in the English and Chinese literature.RESULTS A palpable mass (71.2%), emaciation (54.2%) and pain (54,2%) were common manifestations in renal MFH, Of all the cases, 51 were identified as a storiform-pleomorphic subtype by pathologists. Inconsideration of all the prognosis related factors, the residual tumor and high TNM stage predicted a shortened survival duration, but the symptom of a fever served as a better prognostic factor.CONCLUSION Malignant fibrous histiocytoma which arises from the kidney is a rare pathologic type, and possesses a high tendency towards local recurrence and distant metastasis. Despite the poor prognosis, early detection and radical surgery can prolong survival in selected cases.     

Neurohormonal peptides in ovarian carcinoids: an immunohistochemical study of 81 primary carcinoids and of intraovarian metastases from six mid-gut carcinoids

Eighty-one primary ovarian carcinoids and intraovarian metastases from six mid-gut carcinoids were examined for the presence of tumor cells immunoreactive with antisera raised against various neurohormonal peptides, mostly of gastroenteropancreatic (GEP) origin. Twenty of the primary and two of the metastatic carcinoids contained such tumor cells. The incidence of tumors with any kind of neurohormonal peptide immunoreactive tumor cells was 53% in the trabecular carcinoids, and 42% in the strumal carcinoids, whereas the incidence was much lower (7%) in the insular type. Immunoreactive pancreatic polypeptide (PP), glucagon, enkephalin, and somatostatin were those neurohormonal peptides most commonly observed in the tumor cells of the primary carcinoids. Those less commonly found were substance P, calcitonin, VIP, neurotensin, beta-endorphin, and ACTH. Four metastatic carcinoids were nonreactive with all the antisera used. Cells storing immunoreactive insulin, glucagon, PP, VIP, gastrin, substance P, or enkephalin were found in one of the two remaining metastatic carcinoids; in the other only gastrin-immunoreactive tumor cells were observed. The occurrence and distribution of tumor cells storing the neurohormonal peptides in ovarian carcinoids are discussed in relation to their possible origin in the ovary and to carcinoids in the gut.     

Radiation treatment of primary tracheal cancers

Two cases of primary squamous cell carcinoma of trachea are presented here, One case of low tracheal tumour with supraclavicular lymphnode metastasis presented with air way obstruction and required emergency treatment, is discussed. Second patient with high tracheal tumor which also presented with air way obstruction was managed with radiation therapy after tracheostomy. The management of both cases is described along with review of literature regarding primary tracheal tumors and its management.     

Intracranial osteosarcomas: Report of four cases and review of the literature

Four cases of intracranial osteosarcoma are reported. In three cases the tumors were primary neoplasms arising from the sellar region, base of the occiput and the parieto-occipital area. One case represented osteosarcoma metastatic to the cerebellum, a site not previously described, from a primary tumor at the femur. In addition, the current literature on intracranial osteosarcoma is reviewed.     

Pancreatic islet cell carcinoid: a highly malignant form of carcinoid tumor

A 23-year-old man died four months after being admitted to the hospital because of back pain, 9.9 kg weight loss in 2 months, 8 to 10 loose stools per day, and periodic sweating. The carcinoid syndrome was present but no hypoglycemia. At autopsy, malignant carcinoid tumor was found in the pancreas. No other tumor was found in the usual primary sites for carcinoids. Biochemical findings and clinical data in retrospect support the morphologic diagnosis of pancreatic islet cell carcinoid tumor. Our case and a review of the literature emphasize that metastasizing tumors of the pancreas associated with excessive production of indolic compounds are frequently aggressive and the survival time is short (range 3 months to 4 years; mean 15 months).     

Gastric carcinoids and their precursor lesions. A histologic and immunohistochemical study of 23 cases.

A histologic and immunohistochemical study was carried out in 23 unselected nonantral gastric carcinoids and their precursor lesions classified according to Solcia et al. None of the patients showed Zollinger-Ellison syndrome. Two variants of carcinoids showing distinctive pathologic and pathogenetic characteristics were identified on the basis of presence or absence of associated chronic atrophic gastritis type A (A-CAG). Chronic atrophic gastritis type A was found in 19 cases showing either single or multiple neoplasms, tumor extension limited to the mucosa or submucosa, consistent endocrine cell precursor changes in extratumoral mucosa, and consistent hypergastrinemia and/or G cell hyperplasia. Associated precursor lesions were only hyperplastic in all but two cases with single carcinoids whereas they were also dysplastic in all but one case with multiple carcinoids. The four tumors arising in nonatrophic mucosa were all single, more aggressive, and not associated with extratumoral endocrine cell proliferations or with signs of gastrin hypersecretion. Tumor cells were diffusely immunoreactive for chromogranin A and synaptophysin but usually negative for chromogranin B or HISL-19. Scattered serotonin cells were found in ten carcinoids. They were more frequent in infiltrating than in intramucosal tumors as were the less represented pancreatic polypeptide cells whereas the reverse was found for alpha-subunit-containing cells. These results are of relevance for tumor pathogenesis and may provide the rationale for a less aggressive therapeutic approach in the patients.     

Primary malignant mediastinal germ cell tumors: a study of eleven cases and a review of the literature

From 1957 to 1988 eleven cases of primary mediastinal germ cell tumor were referred to the Peter MacCallum Cancer Institute (PMCI). Four were seminomas, three were mixed germ cell tumors, two were embryonal carcinomas and two were teratocarcinomas. Two of the eleven patients were female. For seminoma, surgical debulking and post-operative irradiation produced the best results. Mediastinal doses ranged from 30 to 37.5 Gy. Local control was achieved in all cases; two patients survive disease-free. The non-seminomatous germ cell tumors showed a significantly poorer survival with only one of seven patients remaining alive in remission at 15 months. One other case of non-seminomatous tumor remains alive but in relapse at 23 months. Attention is focused on the anterior position of primary germ cell tumors in the mediastinum. A review of the literature is presented.     

Carcinoid of ampulla of Vater

Carcinoid tumors of ampulla are rare clinical entities. They form 0.35% of all the gastrointestinal carcinoids. So far, only 109 cases have been reported in the literature, mostly as individual case reports. Since the metastatic potential and the tumor size have no correlation, unlike in duodenal carcinoids, pancreatoduodenectomy is considered the treatment of choice. Here we present a case of carcinoid of ampulla presenting to our department.     

Combined choriocarcinoma and adenocarcinoma of the lung occurring in a man: case report and review of the literature

BACKGROUND: Human chorionic gonadotropin (hCG)-producing large or giant cell carcinoma of the lung is not uncommon, but primary pulmonary choriocarcinoma is an extremely rare entity. Even rarer are cases occurring in males; to date the authors have found only 12 reported cases in the English literature. METHODS: The clinical record of a 61-year-old man who presented with hemoptysis is described. A review of the literature regarding patients with primary pulmonary choriocarcinoma also is reported. RESULTS: Computed tomography scan of the chest demonstrated an expanding thickness of the bullous wall within areas of emphysematous change in the lower lobe of the right lung. Moreover, a new, round tumor near the thickness appeared and rapidly expanded evenly into the surrounding lung tissue. Exploratory thoracotomy revealed the previous tumor to be adenocarcinoma with a small foci of choriocarcinoma, and the new tumor to be a hemorrhage with choriocarcinoma. Because of the pleural dissemination, the patient was treated with chemotherapy. At last follow-up he was alive and well with a gradually increasing serum hCG-beta level in spite of chemotherapy. CONCLUSIONS: Primary pulmonary choriocarcinoma occurring in men is an extremely rare entity with a fatal prognosis. Of the 12 cases reported to date in the English literature, 3 cases of choriocarcinoma with the coexistence of another type of pulmonary carcinoma were reported. To the authors' knowledge the clinical relation between these two types of carcinoma are unknown because all cases to date have been detected at the time of autopsy. Only in the current study case could the clinical course of the disease be followed and pathologic confirmation achieved, although the pathogenesis of the two types of carcinoma could not be determined.     

Synovial sarcoma of abdominal wall: a case report and study of tumor fine structure

A case of synovial sarcoma in the abdominal wall of a 50-year-old Iraqi woman is reported. The ultrastructure of the tumor has been studied. Eight other cases of synovial sarcoma in the abdominal wall reported in the English literature up to 1978 have been reviewed. Synovial sarcomas in the abdominal wall tend to occur with a much greater frequency in women, whereas such tumors occurring in the extremities or the neck are more common in men than woman. No ultrastructural studies of abdominal wall synovial sarcomas could be found in the English literature. The ultrastructural characters of this tumor are similar to those of such tumors arising in the usual sites and the neck. However, the present study has indicated that the light and dark cells reported by earlier workers are not limited solely to the epitheloid areas but are also present in the spindle cell zones.     

Synovial sarcoma of the abdominal wall: a case report and study of its fine structure

A case of synovial sarcoma in the abdominal wall of a 50-year-old Iraq female is reported. The ultrastructure of the tumor has been studied. Eight other cases of synovial sarcoma in the abdominal wall which reported in the English literature up to 1978 have been reviewed. Synovial sarcomas in the abdominal wall tend to occur with a much greater frequency in females in contrast to such tumors in the extremities or the neck which tend to occur with a much greater frequency in males. No ultrastructure studies of abdominal wall synovial sarcomas could be found in the English literature. Ultrastructure characteristics of this tumor were similar to those arising in the usual sites and in the neck. However, the present study has indicated that the light and dark cells reported by earlier workers are not solely limited to the epitheloid areas but are also present in the spindle cell zones.     

原发性气管和主支气管恶性肿瘤的外科治疗

目的 探讨原发性气管和主支气管恶性肿瘤外科治疗的临床经验.方法 对18例原发性气管和主支气管恶性肿瘤患者进行外科手术治疗,其中12例在非体外循环下进行,6例在体外循环下进行.全组气管袖式切除、端端吻合8例,气管下段和隆突切除、隆突重建4例,单纯行肿瘤刮除术4例,右全肺加隆突切除1例,左全肺加隆突切除1例.结果 腺样囊性癌7例,鳞状细胞癌9例,淋巴上皮样癌1例,滤泡型非霍奇金淋巴瘤1例.术后10 d,1例患者因气管切开后气管内大出血而窒息死亡,其他患者术后呼吸困难均有明显改善,近期疗效较好.结论 原发性气管和主支气管恶性肿瘤首先应考虑手术切除,并根据患者的具体情况选择适当的手术方法;手术治疗要兼顾根治性和安全性.     

Goblet cell carcinoid tumors of the appendix: An overview

Goblet cell carcinoid is an enigmatic and rare tumor involving the appendix almost exclusively. Since its identification in 1969, understanding of this disease has evolved greatly, but issues regarding its histogenesis, nomenclature and management are still conjectural. The published English language literature from 1966 to 2009 was retrieved via PubMed and reviewed. Various other names have been used for this entity such as adenocarcinoid, mucinous carcinoid, crypt cell carcinoma, and mucin-producing neuroendocrine tumor, although none have been found to be completely satisfactory or universally accepted. The tumor is thought to arise from pluripotent intestinal epithelial crypt-base stem cells by dual neuroendocrine and mucinous differentiation. GCCs present in the fifth to sixth decade and show no definite sex predominance. The most common clinical presentation is acute appendicitis, followed by abdominal pain and a mass. Fifty percent of the female patients present with ovarian metastases. The histologic hallmark of this entity is the presence of clusters of goblet cells in the lamina propria or submucosa stain for various neuroendocrine markers, though the intensity is often patchy. Atypia is usually minimal, but carcinomatous growth patterns may be seen. These may be of signet ring cell type or poorly differentiated adenocarcinoma. Recently molecular studies have shown these tumors to lack the signatures of adenocarcinoma but they have some changes similar to that of ileal carcinoids (allelic loss of chromosome 11q, 16q and 18q). The natural history of GCC is intermediate between carcinoids and adenocarcinomas of the appendix. The 5-year overall survival is 76%. The most important prognostic factor is the stage of disease. Appendectomy and right hemicolectomy are the main modalities of treatment, followed by adjuvant chemotherapy in select cases. There is some debate about the surgical approach for these tumors, and a summary of published series and recommendations are provided.     

Primary frontal sinus aspergillosis: an uncommon occurrence

Primary frontal sinus aspergillosis is extremely uncommon with only few cases reported in the English literature. Isolated frontal sinus aspergillosis is usually asymptomatic and produces symptoms due to orbital or intracranial involvement. We report two cases of primary frontal sinus aspergillosis, presenting as frontoethmoidal mucocele and mimicking a 'Pott's puffy tumor,' respectively. Forehead swelling produced by frontal sinus aspergillosis will be the first to be reported in the English literature.