Aggressive papillary middle-ear tumor. A clinicopathologic entity distinct from middle-ear adenoma

A 29-year-old woman had a middle-ear mass that resulted in hearing loss and seventh-nerve palsy. The tumor invaded the petrous bone, the mastoid, at least one semicircular canal, and then extended into the posterior fossa. Microscopically, it consisted of complex, interdigitating papillae lined by uniform, cuboidal to low columnar cells. The cells resembled the epithelium of the normal middle ear and middle-ear adenoma, but the papillary architecture distinguished this neoplasm from the latter, nonpapillary tumors. Papillary middle-ear tumors are locally invasive. We propose the term "aggressive papillary middle-ear tumor" (APMET). Nine other examples of APMET have been reported under various diagnostic terms. All have been locally destructive with frequent intracranial invasion. Although none of the tumors has metastasized, one patient died of uncontrolled local disease. For this reason, APMET must be distinguished from nonpapillary middle-ear tumors.     

Dorsal third ventricular cyst: an entity distinct from holoprosencephaly.

The treatment and subsequent developmental progress of six children with dorsal third ventricular cysts are described. This cystic malformation has a radiological appearance which is superficially similar to that of the dorsal cyst of alobar holoprosencephaly, especially when the third ventricular cyst is large. Indeed, previous reports have identified this abnormality as a form of holoprosencephaly. However, careful study reveals that the dorsal third ventricular cyst is a distinct entity both developmentally and clinically. The six patients in this series were effectively treated with shunts, and their subsequent developmental progress was assessed by means of the Prescreening Developmental Questionnaire-Revised as well as the Bayley Scales of Infant Development. The nomenclature and differences between this entity and the holoprosencephalies are reviewed. The authors conclude that dorsal third ventricular cysts have a developmental and clinical course more similar to that of arachnoid cysts than to that of the holoprosencephalies.     

Adenomatous and foveolar gastric dysplasia: distinct patterns of mucin expression and background intestinal metaplasia

Gastric epithelial dysplasia (GED) can be morphologically categorized into adenomatous (or intestinal) and foveolar (or gastric) types. Although limited genetic differences have been demonstrated between these subtypes, the expression of various mucins has not been systematically evaluated in this context. Endoscopic mucosal resections from 69 cases of GEDs were evaluated for the expression of MUC2, MUC5AC, MUC6, and CD10. The results were correlated with morphologic categorization and clinicopathologic parameters. GED was classified as adenomatous, foveolar, or hybrid (showing features of both types), on the basis of histologic evaluation. The neighboring intestinal metaplasia (IM) was also evaluated. An adenomatous morphology was seen in 45%, hybrid type in 33.3%, and a "pure" foveolar type was seen in 21.7% of the cases. Foveolar GED was often depressed/flat on endoscopy and showed a statistically significant association with high-grade morphology (P = 0.046). Immunohistochemistry confirmed the histologic stratification. The foveolar and hybrid types were more often positive for MUC5AC (P = 0.0001 for both) and negative for CD10 (P = 0.019 and 0.016, respectively) as compared with adenomatous GED. High-grade morphology was associated with MUC5AC expression regardless of the morphologic phenotype (P = 0.026). Foveolar (73.3%) and hybrid (86.9%) GEDs were associated more often with IM showing a retained expression of gastric type mucin than adenomatous GED (29%) (P < 0.01 for both). In contrast, adenomatous type (58.1%) of GED was significantly associated with IM showing a complete intestinal phenotype (CD10+) compared with the foveolar (13.3%) and hybrid types (17.4%) of GED (P = 0.005 for both comparisons). In conclusion, our study indicates that foveolar and adenomatous types of GED have distinct clinicopathologic features, mucin profiles, and association with different types of IM.     

C cell adenoma of the thyroid: a rare but distinct clinical entity

Although the benign counterpart of medullary carcinoma of the thyroid has never been indicated in textbooks, we propose that C cell adenoma is a rare but distinct clinical entity. Two patients, a 43-year-old man and a 53-year-old woman, had similar thyroid tumors, both about 4 cm in diameter. The cut surfaces of the resected tumors were indistinguishable from a common microfollicular adenoma of the thyroid. Microscopically, the tumors were uniformly composed of fusiform cells without any follicle formation. Neither amyloid deposition nor calcification was found. Although some kind of C cell tumors were suggested, the exact nature was debatable. However, extremely high levels of calcitonin (1330 and 2065 pg/ml, respectively; normal level, less than 170 pg/ml) were found in the stored sera taken preoperatively. Serum levels of carcinoembryonic antigen (CEA) were normal in both patients. Immunohistochemically, the tumor tissues were positive for calcitonin and neuron-specific enolase but negative for CEA with a monoclonal anti-CEA antibody. No somatostatin, glucagon, or adrenocorticotropic hormone activity was found. It is highly probable that such tumors have not been closely studied and have been regarded as eccentric adenomas of the thyroid or simply as the so-called medullary carcinomas of the thyroid.     

Early gastric cancer: is it a distinct clinical entity?

Of 199 patients with gastric cancer seen at The Montreal General Hospital between 1970 and 1981, 104 were considered to have had a curative resection, and 26 of these were early gastric cancers (EGC). The authors compared early gastric cancers with advanced, but resectable, gastric cancers to determine whether EGC is a distinct entity or a stage in the progressive evolution of gastric cancer. They found that depth of invasion was the primary determinant of outcome, but that there was no discrete cut-off point between the depth of invasion associated with early and with advanced gastric cancers. The pathological features normally associated with a favourable prognosis in gastric cancer, such as absence of lymph-node metastases, an expanding growth pattern, intestinal metaplasia, and well-differentiated histologic features correlated highly with depth of invasion but did not appear to change abruptly between EGC and advanced resectable lesions. The authors conclude that EGC is not a distinct pathological or clinical entity but a stage in the progressive growth of gastric cancer.     

Immunotactoid (microtubular) glomerulopathy: an entity distinct from fibrillary glomerulonephritis?

Immunotactoid glomerulopathy (IT), or alternatively, microtubular glomerulopathy, is a term that has been used by some investigators to encompass those glomerulopathies characterized by the presence of fibrillar or microtubular deposits which are distinguished from amyloid principally by their larger size and lack of reactivity with the Congo red reagent. A case is presented here of IT as it was initially described. The present case, and a review of the literature, suggest that the diagnosis of IT be restricted to those glomerulopathies associated with large, at times organized, microtubular deposits. Terms such as fibrillary glomerulonephritis, or alternatively Congo red-negative amyloidosis-like glomerulopathy, could then be used to describe those patients with smaller, fibrillar glomerular deposits having an appearance closely resembling amyloid. We believe it is possible to separate the entities fibrillary glomerulonephritis and IT on morphological grounds. This is a potentially useful distinction that identifies patients who are likely to have or develop clinical evidence of a lymphoplasmacytic disorder or dysproteinemia, which are associated with deposits of IT, and distinguishes them from patients with fibrillary glomerulonephritis, of whom only a single case has been linked to such clinical findings.     

Basal cell adenoma with myoepithelial cell-derived "stroma": a new major salivary gland tumor entity

The light microscopic, immunohistochemical, and ultrastructural features of a unique variant of tubular-trabecular basal cell adenoma are described. The unusual feature of the six examples reported is the richly cellular "stroma" composed of spindle cells coursing between the anastomosing cords of epithelial tumor cells. Immunohistochemistry of all six cases and electron microscopy of two examples illustrated the biphasic differentiation of the epithelial portion of this form of basal cell adenoma, with a central core of duct luminal cells bordered on either side by one or more layers of modified myoepithelial cells. By light microscopy, the features and arrangement of cells in "stromal" regions of this tumor convey a fibroblastic derivation. However, this population of cells stains strongly for S-100 protein, ultrastructurally displays excessive external lamina production, intercellular junctions, and a growth pattern unlike fibroblasts, and is involved in the formation of extracellular mucinous materials. Such aspects indicate a second population of neoplastic myoepithelial cells in this tumor. Thus, this form of tubular-trabecular basal cell adenoma displays tricellular differentiation and, perhaps, may be considered either a hybrid basal cell adenoma and myoepithelioma or a cellular pleomorphic adenoma.     

Mucous cell adenoma originating from bronchial gland

The clinical and pathological features of a mucous adenoma arising from bronchial gland and manifesting with productive cough and dyspnoea over a period of four years in a 44-year-old male is presented. Origin of the tumour from the proximal part of the bronchial tree necessitated a pneumonectomy with resultant cure.     

Gastric foveolar metaplasia with dysplastic changes in Brunner gland hyperplasia: possible precursor lesions for Brunner gland adenocarcinoma

Cases of adenocarcinomas developed in Brunner gland hyperplasia (BGH) have been sporadically reported. Herein, we report the morphologic spectrum of hyperplastic changes culminating into dysplasia and carcinoma in 722 cases of BGH listed in our files. Fifteen of these cases showed dysplastic changes, with 8 graded as low-grade dysplasia, 5 as high-grade dysplasia, 11 as atypical hyperplasia, and 2 as invasive carcinoma, although each frequently coexisted in the same tumor. In two carcinomas, one had high-grade dysplasia in the mucosa, and another had only atypical hyperplasia. Interestingly, hyperplastic glands around dysplastic foci were associated with gastric foveolar metaplasia and papillary configuration in 13 cases, 11 of which showed a gradual increase in nuclear atypism in the transition from metaplastic to dysplastic glands. All of the metaplastic gastric glands showed diffuse and strong immunopositivity for gastric foveolar mucin (MUC5AC). Immunohistochemical profiles also supported the concept of a continuous spectrum in carcinogenesis from gastric foveolar hyperplasia through atypical hyperplasia or dysplasia and eventually to frank adenocarcinoma. The results of our study suggest, therefore, that dysplastic and/or carcinomatous change does occur in BGH, that they form the continuous morphologic spectrum, and that papillary foveolar metaplasia may be a precursor lesion in the process of carcinogenesis with a background of BGH.     

Chronic recurrent multifocal osteomyelitis: a distinct clinical entity

We reviewed the cases of five children with the diagnosis of chronic recurrent multifocal osteomyelitis (CRMO) and compared and contrasted them to 11 cases of subacute osteomyelitis. Significant differences were found between these two groups in the number of cases with positive biopsy cultures, number of clinical episodes, and number of bones involved. In CRMO, cultures are negative, and recurrent clinical episodes involve different bones at different times. The data indicate that CRMO is a distinct clinical entity, different from subacute osteomyelitis; it is a benign, self-limiting inflammatory disease of bone, and no chronic problems have occurred as a result of CRMO. Restraint in antibiotic treatment and in performing repeated biopsies is indicated in CRMO.     

Deep lobe parotid gland lipoma: an extremely rare entity. A case report

The incidence of lipoma in the parotid gland is very low, and lipomas in the deep lobe of the parotid are extremely rare and seldom considered in the differential diagnosis of deep lobe parotid gland tumours. A deep lobe parotid gland lipoma is presented and discussed.     

Intrathyroidal epithelial thymoma: An entity distinct from squamous cell carcinoma of the thyroid

We report 3 cases of intrathyroidal epithelial thymoma, a clinicopathologic entity distinct from squamous cell carcinoma of the thyroid. Two patients are long-term survivors among 5 postoperative patients with squamous cell carcinoma of the thyroid. One has been free from the disease for 17 years, and the other with local recurrence died of an unrelated disease 18 years after the surgery. The diagnosis in these 2 was corrected to intrathyroidal epithelial thymoma following a review of the histological findings. These 2 tumors consisted of tumor cell nests resembling poorly differentiated squamous cell carcinoma surrounded by many lymphocytes. Features discriminating thymoma from squamous cell carcinoma included the lack of foci of anaplastic carcinoma and papillofollicular tumor, which are seen in squamous cell carcinoma of the thyroid. Two of the 3 patients with squamous cell carcinoma died within 1 year. We have found recently another case of thymoma. The present observations indicate that: thymoma can arise in the thyroid gland presumably from aberrant thymic tissue, mimicking squamous cell carcinoma; and the prognosis of thymoma is favorable. Therefore, it is important to discriminate thymoma from squamous cell carcinoma of the thyroid, which is usually fatal.

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Resumen Se informan tres casos de timoma epitelial intratiroideo, una entidad clínicopatológica diferente del carcinoma escamocelular de la glándula tiroides. Dos de los pacientes son sobrevivientes a largo plazo entre un grupo de 5 pacientes operados por carcinoma escamocelular de la tiroides. Uno se encuentra libre de enfermedad desde hace 17 años, y el otro, con recurrencia local, murió a causa de una enfermedad no relacionada 18 años después de la cirugía. El diagnóstico en estos dos pacientes fué corregido hacia timoma epitelial intratiroideo después de una revisión de la histología. Estos dos tumores consistían de nidos de células tumorales que simulaban un carcinoma escamocelular pobremente diferenciado y rodeado de numerosos linfocitos. Las características que diferencian al timoma del carcinoma escamocelular incluyen la ausencia de focos de carcinoma anaplásico y de tumor papilofolicular, los cuales están presentes en el carcinoma escamocelular de la tiroides. Dos de los tres pacientes con carcinoma escamocelular murieron en el curso de un año. Recientemente hallamos otro caso de timoma.Las observaciones presentes indican que: el timoma puede originarse en la glándula tiroides, presumiblemente a partir de tejido tímico aberrante, y llegar a simular al carcinoma escamocelular; y que el pronóstico del timoma es favorable. Por consiguiente, es importante diferenciar el timoma del carcinoma escamocelular de la tiroides, el cual tiene una evolución usualmente fatal.

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Résumé Trois cas de thymome intrathyroïdien, une entité histologique et clinique différente du cancer épidermoïde de la thyroïde, sont rapportés.Deux malades sur cinq qui avaient été opérés d'un cancer épidermoïde de la thyroïde ont survécu à long terme. Un est en excellent état 17 ans après l'opération, l'autre qui présentait une récidive est mort 18 ans après l'intervention d'une autre affection. A la révision des coupes histologiques les concernant le diagnostic supposé de cancer épidermoïde fut corrigé, en réalité il s'agissait de thymome, la lésion étant caractérisée par des nids de cellules tumorales très proches des cellules peu différenciées des cellules des cancers thyroïdiens épidermoïdes entourés de lymphocytes. Ce qui permet de distinguer le thymome intrathyroïdien du cancer thyroïdien épidermoïde est l'absence de foyer de cancer anaplasique et de tumeur papillofolliculaire. Deux des 3 malades qui présentaient un véritable cancer épidermoïde moururent en moins d'un an. Un autre cas de thymome intrathyroïdien vient d'être observé.Ces observations permettent d'affirmer que: le thymome peut se développer au sein de la thyroïde probablement au niveau d'un foyer ectopique du thymus et simuler le cancer épidermoïde, et le pronostic des thymomes est favorable et qu'il est par conséquent très important de distinguer les deux affections.

     

Villous adenoma of the extrahepatic biliary tract: a rare entity

Patients presenting with large obstructing extrahepatic biliary tumors often are presumed to have cholangiocarcinoma and are labeled with a grim disease with a poor prognosis, given little hope for a cure, and may actually opt for palliative care only. In some instances, however, the diagnosis is that of biliary adenoma (benign until it undergoes malignant degeneration), which can be confirmed via resection and pathologic evaluation of the lesion. Removal of the tumor in its benign stage then provides curative treatment of the obstructing lesion with excellent patient recovery and overall prognosis. We present a rare instance of observation of the presence of high-grade dysplasia in a large villous adenoma arising from the left hepatic duct with relief of biliary obstruction and curative resection.     

Pelvic fracture in geriatric patients: a distinct clinical entity

BACKGROUND: The purpose of this study was to describe differences in demographics, injury pattern, transfusion needs, and outcome of pelvic fractures in older versus younger patients. METHODS: This was a retrospective registry review of all patients with pelvic fractures admitted directly from the scene between January 1998 and December 1999. RESULTS: We cared for 234 patients with pelvic fractures during the study period. Mean age was 37.2 years, 51% were men, and mean Injury Severity Score (ISS) was 19. Overall mortality was 9%. Eighty-three percent were under the age of 55 years and 17% were older than 55 years. Severe pelvic fractures (AP3, LC3) were more common in young patients (p < 0.05). Admitting systolic blood pressure was lower and heart rate higher, although ISS was not different between the two age groups. Older patients were 2.8 times as likely to undergo transfusion (p < 0.005), and those undergoing transfusion required more blood (median, 7.5 units vs. 5 units). Older patients underwent angiography more frequently and were significantly more likely to die in the hospital even after adjusting for ISS (p < 0.005). This was most marked with ISS 15 to 25. Lateral compression (LC) fractures occurred 4.6 times more frequently in older patients than anteroposterior (AP) compression, and 8.2 times more frequently in those older patients undergoing transfusion as compared with AP compression. Ninety-eight percent of LC fractures in older patients were minor (LC1,2). However, older patients with LC fractures were nearly four times as likely to require blood compared with younger patients. CONCLUSION: In older patients, pelvic fractures are more likely to produce hemorrhage and require angiography. Fracture patterns differ in older patients, with LC fractures occurring more frequently, and commonly causing significant blood loss. The outcome of older patients with pelvic fractures is significantly worse than younger patients, particularly with higher injury severity. Recognition of these differences should help clinicians to identify patients at high risk for bleeding and death early, and to refine diagnostic and resuscitation strategies.     

Neuroendocrine differentiated carcinomas of the breast: a distinct entity

Some primary carcinomas of the breast have been classified as neuroendocrine. In this paper we report on 12 cases of neuroendocrine carcinoma of the breast displaying common and uncommon histological features. MATERIALS AND METHODS: All patients were aged from 43 to 79 (median 66.5) years. The tumors were unilateral and ranged from 0.8 to 7 cm (median 2.35 cm) in diameter. Four tumors were classified as solid cohesive, two as solid papillary, one as Merkel cell-like, two as cellular mucinous, two as cellular mucinous with micropapillary features and one as cellular mucinous with prominent signet ring cells. The tissues were fixed in formalin and routinely processed. All materials were stained with PAS, Alcian blue and also hematoxylin and eosin. Immunohistochemical examination was carried out by the avidin-biotin method using the following antibodies: estrogen receptor protein, progesterone receptor protein, p53, cerbB-2, Ki67, neuron-specific enolase, chromogranin, and synaptophysin. RESULTS: In all cases the tumor cells were stained positively with NSE. Synaptophysin and chromogranin positivity was detected in 11 and five cases, respectively. The prominent histological features were: extensive intraductal growth, pseudorosettes and palisades, low-grade cytologic atypia, plasmacytoid appearance of the tumor cells, and intracellular and extracellular mucin. CONCLUSION: Neuroendocrine-differentiated carcinoma of the breast is a rare and distinct category with different histological subtypes.