Medical care in 7 Chilean cities

Sclerosing mucoepidermoid carcinoma with eosinophilia (SMECE) is a recently recognized malignant neoplasm of the thyroid gland. Two additional cases of this condition which occurred in a 70-year-old woman and a 69-year-old woman are presented. The case of the 70-year-old woman (patient 1) is the first report of distant metastasis, besides lymph node metastasis, for this type of tumor. The patient initially presented with a thyroid mass, and the thyroid gland with surrounding cervical lymph nodes was removed. Because of focal keratin "pearl" formation, the tumor was misinterpreted as a metastatic squamous cell carcinoma to the thyroid. Approximately 4 years later, the patient developed a left supraclavicular mass and lung densities. A pathological fracture of the right humeral head followed, and the left supraclavicular mass recurred along with newly developed subcutaneous nodules on the chest wall and arm. Open lung and bone biopsies revealed metastatic SMECE, which was morphologically identical to that of the thyroid mass. The 69-year-old woman (patient 2) had, in 1983, undergone thyroidectomy with left radical neck dissection; this had been diagnosed as follicular carcinoma of the thyroid with lymph node involvement. After multiple isolated lymph nodes metastases, the patient developed locally extensive, recurrent tumor that showed microscopic features of SMECE. Review of the previous thyroid tumor and lymph nodes revealed the same type of histology. To our knowledge, only a single report containing eight cases of this distinctive carcinoma of the thyroid has been published. Herein we describe characteristic morphological features of two additional cases of this rare malignancy, one with distant metastasis, and we review the related literature.     

Aspiration cytology of ectopic cervical thymoma mimicking a thyroid mass. A case report

BACKGROUND: Ectopic cervical thymoma, first described in 1941 by Boman, is an uncommon tumor of the neck displaying the same histologic features as mediastinal thymoma. Since it is commonly located in the anterolateral part of the neck or is subjacent to or inside the lower pole of the thyroid, the mass is often confused as being of thyroid origin. CASE: A 68-year-old female presented with dyspnea and an anterior neck mass found on routine chest roentgenography. The thyroid scan showed a cold nodule in the lower pole of the left part of the thyroid. Fine needle aspiration (FNA) cytology revealed large numbers of small lymphocytes with hyperchromatic nuclei and frequent clumping pattern in the pale, eosinophilic, fluid background. A few clusters of epithelial cells without atypism were interpreted as thyroid follicular cells. The overall cytologic features were misinterpreted as malignant lymphoma of the thyroid. However, the histologic diagnosis was thymoma, predominantly cortical type. CONCLUSION: The ectopic cervical thymoma is sometimes misdiagnosed as Hashimoto's thyroiditis, anaplastic carcinoma and malignant lymphoma of thyroid on FNA cytology or frozen diagnosis due to its rarity. Therefore, the differential diagnosis of a neck mass showing a variable composition of lymphocyte and epithelial component in a pale, eosinophilic, fluid background should also include ectopic cervical thymoma, especially in elderly females.     

Testicular epidermoid cysts in prepubertal children: case report and review of the world literature

Epidermoid cysts of the testes are rare, benign lesions that account for approximately 1% of all testicular tumors. They present most often between the second and fourth decade of life, and have been reported in prepubertal children rarely. The authors report the clinical and sonographic findings in a 4 1/2-year-old child with a testicular epidermoid cyst, and they analyze the 22 prepubertal cases found in the world literature. As described below, the clinical and sonographic features of this tumor are not specific for an epidermoid cyst and do not preclude a teratomatous or malignant neoplasm. This case adds to the varied sonographic appearance of prepubertal testicular epidermoid cysts. The lack of either unique clinical or sonographic features would suggest that extreme caution be used if local excision is considered for a patient with presumed epidermoid cyst of the testicle.     

Analysis of case-control studies of the efficacy of screening for cancer: How should we deal with tests done in persons with symptoms?

OBJECTIVE: The congenital occurrence of malignant rhabdoid tumor (MRT) is rare. Initial presentation of congenital MRT as a skin tumor is even more rare. We report 2 cases in which a cutaneous nodule appeared prior to the discovery of the primary tumor. METHODS: Histologic, ultrastructural, and immunohistochemical studies were performed on skin nodules from 2 neonates. Other congenital examples of MRT in the literature were reviewed. RESULTS: Both neonates were male and both were fullterm. Skin nodules were noted at birth. The tumors were located on the right neck in case 1 and on the right back in case 2. The primary tumors were later found in the kidney and the right chest wall, respectively. The skin biopsy of the first case had been interpreted as undifferentiated sarcoma, and MRT was diagnosed after the renal tumor was examined. In the second case, a correct diagnosis was promptly made based on the skin biopsy owing to experience with the first case. CONCLUSIONS: Congenital MRT is very uncommon and may present as a skin tumor at birth. Since MRT exhibits various histologic patterns, the diagnosis should be made by a complete pathologic study with proper clinical correlation. To our knowledge, the unique mode of manifestation in these 2 cases is described for the first time. We suggest that MRT should be considered when making the differential diagnosis in such a clinical setting.     

Hypercalcaemia in patients with newly diagnosed tuberculosis in Malaysia

A giant hidradenocarcinoma presented by a 75-year-old female is reported. The patient had a malignant transformation within a nodular hidradenoma involving the right postauricular area, which was treated by mass removal and a right radical neck dissection with a free-flap covering. Malignant hidradenocarcinoma is the least common adnexal tumor of uncertain origin. They are usually malignant from their inception, but some develop from a benign counterpart. To the authors' knowledge, only three cases have been reported previously. Two histologically distinct components were seen in this tumor: (i) typical nodular hidradenoma, which constituted a small part of the tumor; and (ii) carcinoma with areas of transition. The secretory cells of hidradenocarcinoma showed decapitation secretion on light and electron microscopic observations, which is evidence of apocrine differentiation. Histologically, this case was concluded as a hidradenocarcinoma arising from a long-standing nodular hidradenoma. A literature review is presented and the histological, immunohistochemical and ultrastructural features are described.     

A gene predisposing to familial thyroid tumors with cell oxyphilia maps to chromosome 19p13.2

Familial nonmedullary thyroid cancer (FNMTC) is a clinical entity characterized by a phenotype more aggressive than that of its sporadic counterpart. Families with recurrence of nonmedullary thyroid cancer (NMTC) have been repeatedly reported in the literature, and epidemiological data show a very high relative risk for first-degree relatives of probands with thyroid cancer. The transmission of susceptibility to FNMTC is compatible with autosomal dominant inheritance with reduced penetrance, or with complex inheritance. Cases of benign thyroid disease are often found in FNMTC kindreds. We report both the identification of a new entity of FNMTC and the mapping of the responsible gene, named "TCO" (thyroid tumors with cell oxyphilia), in a French pedigree with multiple cases of multinodular goiter and NMTC. TCO was mapped to chromosome 19p13.2 by linkage analysis with a whole-genome panel of microsatellite markers. Interestingly, both the benign and malignant thyroid tumors in this family exhibit some extent of cell oxyphilia, which, until now, had not been described in the FNMTC. These findings suggest that the relatives of patients affected with sporadic NMTC with cell oxyphilia should be carefully investigated.     

Cerebellopontine angle lymphoma: a case report and review of the literature

BACKGROUND: Although malignant lymphomas of the central nervous system have been reported to be increasing in frequency, cerebellopontine (CP) angle lymphoma is rare and only 13 cases have been reported previously in the literature. CASE PRESENTATION: A 63-year-old woman had progressive dizziness and nausea for 2 months. Computed tomography scanning and magnetic resonance imaging (MRI) revealed a mass lesion in the left CP angle, that was compressing the lateral-dorsal aspect of the pons and the fourth ventricle. This tumor was avascular on angiography. The tumor was surgically removed through a left lateral suboccipital approach. It was considered to arise from the subarachnoid space of the CP angle cistern. For some reason, the histologic diagnosis was not definitively made, and therefore radiation therapy was not planned. The tumor recurred within 50 days after the tumor excision. Surgical excision of the recurrent tumor was performed again. The histologic diagnosis was B-cell type malignant lymphoma. Radiation therapy was performed. In the 27 months since irradiation, a recurrent tumor has not been detected on MRI. CONCLUSIONS: Although erosion and expansion of the internal auditory canal suggest an acoustic neurinoma, CP angle lymphoma can, in rare circumstances, erode the internal auditory canal. There are three distinct patterns in which malignant lymphomas occupy the CP angle: (1) an extra-axial CP angle lymphoma, (2) an intra-axial lymphoma extending to the CP angle, and (3) a leptomeningeal lymphoma presenting as a CP angle lesion. Although malignant lymphomas rarely occupy the CP angle, it should be considered in the differential diagnosis of CP angle tumors. It is desirable to obtain a frozen section in all CP angle tumors during surgery to identify the tumor, because aggressive removal is not necessary, but radiation therapy should additionally be performed for malignant lymphomas.     

A case of malignant strumal carcinoid

A malignant strumal carcinoid with widespread metastases resembling a well-differentiated thyroid follicular carcinoma is described. Strumal carcinoid is a rare ovarian tumor which usually behaves in a benign manner. Only one malignant case has been reported in the literature and in that case the carcinoid element metastasized. The clinical, histological, and immunohistochemical findings of the current case together with the relationship between strumal carcinoids and struma ovarii are discussed.     

Malignant carcinoid tumor and synchronous malignant extraadrenal paraganglioloma

Gastrointestinal carcinoid tumor is often considered the most common neuroendocrine tumor of the small intestine. The overall incidence of 1% in the general population is quite low. Extraadrenal paragangliomas are rarer still, and the incidence of both of these tumors in the malignant state is exceedingly rare. This article describes the case of a patient who had both a malignant carcinoid tumor as well as a malignant retroperitoneal paraganglioma occurring synchronously. A review of the literature concerning these tumors is also presented.     

Improved survival with resection after transcatheter arterial chemoembolization (TACE) for unresectable hepatocellular carcinoma

Three patients with malignant blue nevus are reported-one on the right side of the body, one on the right arm, and one on the face. The criteria and difficulty of histopathological diagnosis are considered as well as the differential diagnoses for this tumor. The therapy is described, and the possible relations between malignant blue nevus and certain other tumors (e.g., atypical or locally aggressive cellular blue nevus) are explored. A review of the literature reveals that there is current disagreement about the exact prognosis for these tumors and indicates the need to collect data for all patients observed.     

Genotator: a workbench for sequence annotation

The clinical, pathological, and immunohistochemical features of six cases of metastatic neuroendocrine and carcinoid tumors to the thyroid simulating medullary thyroid carcinoma (MTC) are described. The patients were women between the ages of 24 and 70 years who, without symptoms or significant past medical histories, presented with either a single mass or multiple thyroid nodules. The primary source of the tumor was only discovered on follow-up. Two of the neoplasms were classical carcinoid tumors, one was a carcinoid predominantly composed of large cells, another showed a prominent oval to spindle cell component, and the two remaining cases were atypical carcinoid/high-grade neuroendocrine carcinomas. The immunohistochemical profile was inconsistent with MTC in that all tumors were negative for calcitonin and only two were focally positive for carcinoembryonic antigen (CEA). A variable pattern of staining for other neuroendocrine and epithelial markers was obtained in each case. Despite the morphologic and immunohistochemical similarities with MTC, the diagnosis of a metastatic neuroendocrine tumor to the thyroid should be favored in the presence of a predominantly interstitial pattern of spread; occurrence of multiple tumor foci; folliculotropism; rosette formations with lumen and cuticular borders; and lack of immunoreactivity for calcitonin and CEA. The differential diagnosis between MTC and metastatic neuroendocrine carcinoma to the thyroid is of importance because of the vast differences in treatment and prognosis.     

Two-dimensional electrophoretic analysis of membranous protein from human thyroid tissues and cancer cell lines

Thyroid neoplasm is the most commonly encountered neoplastic disorder in endocrine clinics. Thyroid scan, ultrasonography, and fine needle aspiration cytology (FNAC) are used as diagnostic tools to differentiate a malignant nodule from a benign lesion. There are certain limitations and pitfalls in FNAC, especially in the diagnosing of follicular tumors. The lack of characteristic findings or a specific tumor marker are the most common problems in the preoperative diagnosis of thyroid follicular carcinoma. Although serum thyroglobulin level has been used as a tumor marker for post-operative, well-differentiated thyroid cancer, the assay cannot be used for preoperative diagnosis of thyroid carcinoma. In this study, various thyroid tissues and cancer cell lines including CGTH W-1, CGTH W-3, RO 82 W-1, SW 579 cell lines were used for the investigation of tumor markers. Specific spots were identified in the area near the 60 kDa molecular mass protein and isoelectric point (pI) 5.9 of the CGTH W-1 cell line. These spots could not be found in the papillary or anaplastic thyroid cancer cell lines. Another spot with a molecular weight of about 9.8 kDa with a low pI of 4.8 was present in the CGTH W-1 and RO 82 W-1 cell lines. This spot appeared to be a tumor marker of follicular cancer cells. This spot could not be found in the papillary and anaplastic cancer cell lines and other benign thyroid tissues. Specific proteins that were identified in this study may be useful as tumor markers for follicular thyroid carcinoma.     

Significance of nondiagnostic fine-needle aspiration of the thyroid

BACKGROUND: Fine-needle aspiration biopsy (FNAB) has been shown to be rapid and cost effective in the evaluation of thyroid nodules. The significance of nondiagnostic (unsatisfactory) FNAB is uncertain, however. METHODS: We reviewed 345 consecutive thyroid FNABs and identified 59 patients with initially unsatisfactory specimens. These patients had follow-up to determine whether their thyroid nodules proved to be malignant. RESULTS: Three patients (5.1%) were found to have organ-confined papillary carcinoma of the thyroid, the largest tumor mass measuring 1.2 cm. Six patients (10.2%) had benign adenomas. CONCLUSIONS: In most cases of initially nondiagnostic FNAB of a thyroid nodule, neoplasia is not found subsequently. A minority of cases may still harbor malignancy. None of our patients in whom repeated FNA was either nondiagnostic or suggestive of benign disease were ultimately found to have a malignancy.     

Pediatric liver tumors

An infant or child who presents with a large intrahepatic mass will most likely have a malignant tumor. In children, benign tumors constitute only 30% of liver tumors and most are vascular in origin. Treatment of benign vascular tumors is conservative and seldom surgical. Hepatoblastoma is the most common malignant tumor followed by hepatocellular carcinoma. Treatment of malignant tumors is based on a combination of surgery and chemotherapy. Children with hepatic malignancies that can be resected have an excellent prognosis. Other rare benign and malignant tumors of the liver do occur and surgery plays a critical role in management.     

The firm salivary mass in children

The firm salivary gland mass arising in a child is uncommon, representing only 3.2% of all such salivary tumors at the University of Iowa. However, the fact that 57.1% of these tumors are malignant demands thorough diagnostic evaluation by the head and neck surgeon. Histologic diagnosis is strongly recommended. In view of the fact that most of these tumors have a favorable prognosis, lateral parotid lobectomy or total submandibular gland excision represented both an effective diagnostic and adequate therapeutic procedure for benign and low-grade malignancies. High-grade malignancies necessitate adjunctive therapy which is based on the biologic behavior of the particular histologic type.     

B-lymphoproliferative disorders in patients with hepatitis C virus infection

Blastomycosis is an endemic systemic fungal infection that usually involves the lungs and superficial skin. Although head and neck involvement has been reported in the literature, no previous cases of neck mass resulting from direct extension of a pulmonary lesion have been published. We encountered an immunocompetent 31-year-old woman with a rapidly enlarging subcutaneous neck mass and a chronic upper lung infiltrate. Imaging studies showed contiguity between both lesions. Blastomyces dermatitidis was recovered from the sputum, and typical yeast was observed in fungal stains of needle aspirate from the neck mass. The patient responded favorably to a 6-month course of itraconazole. Blastomycosis should be considered in patients with subcutaneous neck masses in areas where this disease is endemic.     

Persistence of multiple serovars of Neisseria gonorrhoeae

The nm23 gene that encodes nucleoside diphosphate (NDP) kinase has been proposed as a candidate tumor metastasis suppressor in rodent experimental carcinoma models and several types of human carcinomas. The present studies were designed to investigate, by immunohistochemical analysis, whether thyroid tissues express the nm23-H1/NDP kinase and, if so, whether the nm23-H1/NDP kinase expression is correlated to tumor metastasis suppressor potential in thyroid tumors. We found that normal thyroid epithelial cells were stained weakly but homogeneously by mouse monoclonal anti-nm23-H1/NDP kinase antibody. The staining intensity of the nm23-H1/NDP kinase in benign thyroid tumors tended to be weaker than that in normal thyroid tissues. In contrast, a series of malignant thyroid tumors expressed differently the nm23-H1/NDP kinase: the intensity of the nm23-H1/NDP kinase staining was stronger in 22 of 49 malignant tumors (45%), similar in 24 (49%), and weaker in 3 (6%) compared to that in normal tissues. The comparison of the staining intensity of the nm23-H1/NDP kinase in primary lesions of tumors with lymph node metastases and those without metastases revealed no statistically significant difference. In addition, there was also no significant difference in the nm23-H1/NDP kinase staining intensity of primary and metastatic lymph node lesions of tumors with lymph node metastases. These data indicate that the nm23-H1/NDP kinase may not be a good predictive marker for tumor regional metastatic potential in malignant thyroid tumors. We conclude that in thyroid tumors the nm23-H1/NDP kinase expression may be dissociated from metastasis suppressor activity.     

Basic fibroblast growth factor in serum and urine of patients with head and neck cancer

Between 1996 and 1997 serum and urine levels of basic fibroblast growth factor (b-FGF) in patients with head and neck cancer were measured to answer the following questions: i) Are increased levels of b-FGF in serum and urine detectable in patients with malignant head and neck tumors? ii) Do these parameters correlate with tumor stage and differentiation of tumors? iii) Is there an association between growth behaviour (local or metastatic growth) and b-FGF levels in serum and/or urine? Eighty-nine patients with head and neck cancer as well as 45 patients with diseases unrelated to cancer were investigated. Detectable levels of b-FGF were found in the serum and urine of patients with malignant head and neck tumors. In addition, there was a significant correlation between tumor size and b-FGF levels in either serum or urine. No association of b-FGF concentrations with degree of histologic differentiation and tumor growth behaviour was observed. The results of this study demonstrate that b-FGF levels are elevated in serum and urine of patients with head and neck cancer. These findings suggest an involvement of b-FGF in the formation of solid tumors.     

Inflammatory myofibroblastic tumor in children

The authors presented the cases of two children with inflammatory myofibroblastic (IMF) tumor and reviewed the literature to facilitate the preoperative recognition, delineate the clinical features, and describe the natural history of this entity. The first child had IMF tumor arising from the mesentery of the small intestine. He presented with an abdominal mass associated with severe inflammatory response manifested by fever, impaired growth, thrombocytosis, and microcytic, hypochromic anemia. After surgical resection, his fever resolved and his growth rate and the laboratory abnormalities normalized. Five months after initial diagnosis, the fever, anemia, and thrombocytosis recurred along with two tumors arising from the omentum and the abdominal soft tissue. After the second surgery, he remains free of recurrent disease for 30 months. The second child presented with a lung mass that was radiologically indistinguishable from pulmonary sequestration. After surgical resection, she remains free of recurrent disease for 18 months. IMF tumor should be considered in any solid tumor that occurs in association with a chronic inflammatory response. IMF tumor should also be considered in the differential diagnosis of pulmonary sequestration.