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C S Heffess

Publications and source records attributed to C S Heffess.

16 recordsLinked to original sources
PubMed1998

Thyroid papillary carcinoma of columnar cell type: a clinicopathologic study of 16 cases.

BACKGROUND: Thyroid papillary carcinoma of columnar cell type is considered an uncommon histologic subtype of papillary carcinoma characterized by its morphologic features and purportedly aggressive biologic course. METHODS: Sixteen cases of thyroid papillary carcinoma of columnar cell type were identified from the Endocrine Tumor Registry at the Armed Forces Institute of Pathology and the Washington Hospital Center. Clinical records and follow-up were available in all cases. Paraffin blocks were available for histochemical and immunohistochemical studies in 15 of the 16 cases. RESULTS: Of the 16 cases reported, 13 patients were female and 3 were male. The ages ranged from 16-76 years (average, 47 years; median, 40 years). An asymptomatic neck mass was the most common clinical presenting symptom. Macroscopically, the tumors varied from circumscribed or encapsulated to infiltrative, ranging in size from 1.5-6.5 cm. Histologically, the tumors had diverse growth patterns, including papillary, solid, microfollicular, and cribriform. A common pattern was the presence of markedly elongated follicles arranged in parallel cords. Colloid-filled follicles could be found, at least focally, in all cases. The characteristic histologic appearance included the presence of elongated cells showing nuclear stratification. Other features included the presence of vacuolated-appearing cells, spindle-shaped cells, and squamoid nests. Limited areas in the tumors showed morphologic features typical of thyroid papillary carcinoma. In 14 of the cases, the tumor was encapsulated, showed limited invasive growth, or was a microscopic tumor. In two of the cases, there was extrathyroidal invasion. Immunohistochemical studies showed consistent reactivity with cytokeratin and vimentin; varied reactivity with thyroglobulin, epithelial membrane antigen, carcinoembryonic antigen, and LeuM1; and no reactivity with calcitonin or chromogranin. Treatment was by surgical resection; supplemental radioactive iodine therapy was administered in eight patients. Follow-up was available for all patients, 13 of whom (81%) were alive and free of disease or had died of unrelated causes over periods ranging from 2-11 years (average, 5.8 years). All 13 of these patients had tumor confined completely to the thyroid gland. One patient died 4 months after diagnosis secondary to sepsis. Two patients (17%) had aggressive biologic courses. In both patients there was extrathyroidal invasion. One of these patients died of metastatic disease to the lungs 3 years after diagnosis; the other was alive with bilateral pulmonary metastases 9 years after the diagnosis. CONCLUSIONS: The findings of the current study indicate that thyroid papillary carcinoma of columnar cell type is a distinct morphologic type but not a distinct clinical type of thyroid papillary carcinoma. The biologic behavior of this tumor is predicated on clinical stage, with the presence or absence of extrathyroidal invasion being the single most important parameter. Treatment of patients with these tumors should be based on the clinical stage and not on the morphologic appearance.

Adolescent↗
PubMed1997

Primary smooth muscle tumors of the thyroid gland.

BACKGROUND: Primary smooth muscle tumors of the thyroid gland are rare. To date, there are few cases reported of primary thyroid leiomyomas and leiomyosarcomas. METHODS: One leiomyoma and four leiomyosarcomas arising within the thyroid gland were identified in the files of the Endocrine Tumor Registry of the Armed Forces Institute of Pathology. Histologic and immunohistochemical features were reviewed and follow-up obtained. RESULTS: The patients included 2 females, ages 56 and 64 years, and 3 males, ages 45, 68, and 83 years. The patients presented with a mass in the thyroid gland that had increased in size over a number of months. All the tumors originated within a single lobe of the thyroid gland and measured from 1.1 to 9 cm in greatest dimension. Histologically, there was a fascicular pattern of growth comprised of spindle-shaped cells with blunt-ended nuclei. The leiomyoma was encapsulated, cytologically bland, and amitotic; the leiomyosarcomas were invasive with increased cellularity, pleomorphism, a high mitotic rate, necrosis, and hemorrhage. Immunohistochemical staining showed reactivity with vimentin, smooth muscle actin, muscle specific actin, and desmin. The patient with the leiomyoma was alive without evidence of disease 11 years after the initial presentation, with surgical resection as the only treatment. Three of the patients with leiomyosarcomas were dead within 2 years of diagnosis, in spite of aggressive therapeutic intervention. The remaining patient was still alive 10 months after initial presentation with multiple lung metastases. CONCLUSIONS: Smooth muscle tumors of the thyroid gland are distinctive tumors. Leiomyosarcomas can be distinguished from anaplastic carcinoma, although patient outcome is uniformly unfavorable.

Aged↗
PubMed1997

Pancreatic mucinous cystic neoplasm with sarcomatous stroma: a report of three cases.

Three patients with pancreatic mucinous cystic neoplasms having a sarcomatous stroma are reported. The tumors occurred in two women and one man, aged 48, 66, and 67 years, respectively. Symptoms included epigastric pain or a palpable abdominal mass or both. Radiographically, all the tumors were large, multicystic, and located in the tail of the pancreas. Histologically, the cystic component was lined by mucin-producing, columnar to cuboidal epithelium composed of benign to atypical to overtly malignant cells (cystadenocarcinoma). Immunohistochemistry showed the epithelial component of all three tumors to be positive with cytokeratin, epithelial membrane antigen, carcinoembryonic antigen, and the pancreatic epithelial mucin antibodies CA 19-9 and DUPAN 2. In all three cases, an undifferentiated malignant spindle cell (sarcomatous) stroma was intimately associated with the epithelial component. The sarcomatous component showed variable reactivity with vimentin (all three cases), muscle-specific and smooth-muscle actin (all three cases), S-100 protein (1/3), Leu-7 (one case), and estrogen and progesterone receptors (in two cases). Two patients died with widespread abdominal disease within 15 months of diagnosis, one of whom had an omental metastasis entirely composed of the sarcomatous stroma. The third patient was alive and free of disease at 16 months after diagnosis. Our findings document the existence of a sarcomatous component in pancreatic mucinous cystic neoplasms; this component appears to be responsible for the highly malignant behavior of the tumors.

Aged↗
PubMed1996

Intraductal oncocytic papillary neoplasms of the pancreas.

We describe the clinical and pathologic features of 11 intraductal oncocytic papillary neoplasms of the pancreas, a hitherto unrecognized tumor. The patients were six men and five women, and most of the tumors were in the head (head: body/tail = 8:3). The mean patient age was 62 (range, 39-78), and the average tumor size was 6 cm. Grossly the tumors exhibited mucin-filled cysts containing nodular papillary projections. Dilated ducts communicating with the main tumor were sometimes noted. Microscopically the cystic structures appeared to represent dilated ducts containing intraductal tumor. The tumors were characterized by variably complex, arborizing papillary structures. The papillae had thin, delicate fibrovascular cores with focal myxoid changes and were lined by stratified oncocytic cells. Goblet cells and intra-epithelial mucin-containing lumina were present, the latter resulting in a characteristic cribriform pattern. The exuberance of the epithelial proliferation varied from case to case and between different regions within individual tumors; solid sheets of cells were often identified. Although the degree of cytologic atypia was not generally severe, the complexity of the architecture justified a designation of intraductal oncocytic papillary carcinoma in 10 of the 11 cases. In nine cases the tumor was entirely intraductal; one case exhibited focal microinvasion and another showed widespread invasive carcinoma, the invasive elements appearing cytologically similar to the intraductal papillary components. The oncocytic cells stained positively with phosphotungstic acid hematoxylin and Novelli stains. Immunohistochemically, all cases stained positively for B72.3, and five cases showed focal, weak luminal membrane staining for carcinoembryonic antigen. Ultrastructurally many of the cells were packed with mitochondria, and mucin was also identified. Seven patients were alive and free of tumor from 1 month to 3 years (average, 1 year) after resection. Two patients died postoperatively. The remaining two patients died with no evidence of disease at 2.5 and 5 years, the latter following a recurrence at 2.5 years. We conclude that intraductal oncocytic papillary neoplasm is a distinctive pancreatic tumor that is usually intraductal but may develop invasive carcinoma and should be treated with complete resection.

Adenocarcinoma, Mucinous↗
PubMed1996

Langerhans cell histiocytosis of the thyroid: a series of seven cases and a review of the literature.

Langerhans cell histiocytosis is a rare disorder, with a few reports describing isolated thyroid gland involvement. We report seven cases, which included four females and three males ranging in age from 2 months to 55 years, with a median age of 37 years. Histologically, the cases demonstrated either diffuse or focal involvement of the thyroid gland by Langerhans cell histiocytes, characterized by bean-shaped, lobated, folded nuclei. In association with the histiocytic infiltrate, there was a prominent eosinophilic cellular component, as well as destruction of the thyroid follicles. All cases occurred in a background of lymphocytic thyroiditis. One case demonstrated adenomatoid nodules, whereas another had a microscopic papillary carcinoma. Immunohistochemical staining demonstrated positive reactivity with S-100 protein, lysozyme, and KP-1. Four patients with isolated thyroid disease, treated by surgical resection alone, are alive without systemic disease from 3 to 19 years after initial presentation. The three patients with systemic disease died within 1 year of the initial diagnosis with disease-related complications. Localized disease portends a favorable prognosis as compared to the thyroid involvement as part of systemic disease.

Adult↗
PubMed1995

Characteristics of follicular tumors and nonneoplastic thyroid lesions in children and adolescents exposed to radiation as a result of the Chernobyl disaster.

BACKGROUND: In addition to the previously reported increase in incidence of thyroid carcinomas in Belarussian children after the Chernobyl disaster in April, 1986, benign thyroid lesions were also found to be increased in the exposed population. METHODS: A total of 60 follicular neoplasms and benign nonneoplastic thyroid lesions arising after the Chernobyl disaster in children and adolescents of 7 to 18 years of age were studied. RESULTS: The primary diagnoses in this series were follicular carcinoma in 1 (2%) case, follicular adenoma in 9 (15%), cystic adenomatoid nodule with papillae in 18 (30%), multinodular goiter in 18 (30%), diffuse hyperplasia in 2 (3%), diffuse hyperplasia with atypia and nodularity in 5 (8%), lymphocytic thyroiditis in 6 (10%), and thyroid cyst in 1 patient (2%). Additional histologic changes in thyroid glands from these patients were similar to those reported after radiation exposure, and included perifollicular fibrosis (72%), focal epithelial hyperplasia (73%), colloid accumulation (47%), follicular atrophy (33%), and cellular atypia (25%). Vascular abnormalities were found more often (75%) than previously reported in the thyroid gland after irradiation, and had a somewhat different appearance. They affected primarily medium-size arteries and were characterized by damage of the internal elastic lamina in addition to intimal fibrosis. CONCLUSIONS: The first case of thyroid follicular carcinoma in the exposed Belarussian children was diagnosed after a latent period of 6.5 years, as compared with 4 years of minimal latency for post-Chernobyl papillary carcinomas. Among benign thyroid lesions, cystic adenomatoid nodules of papillary type and diffuse hyperplasia with cellular atypia and nodularity seem to be commonly associated with radiation exposure to the thyroid gland.

Adenocarcinoma, Follicular↗
PubMed1995

Primary mucoepidermoid carcinoma of the thyroid gland: a report of six cases and a review of the literature of a follicular epithelial-derived tumor.

Primary mucoepidermoid carcinomas of the thyroid gland are uncommon tumors of low-grade malignant potential. We report six cases of thyroid mucoepidermoid carcinoma, four occurred in women and two in men with an age range of 29 to 57 (median, 46 years). The clinical presentation was that of a painless mass. Radiographic studies showed a single, solid, "cold" nodule in either the right or left lobe, or isolated to the isthmus. There was no history of a mucoepidermoid carcinoma developing in more typical locations (eg, salivary gland) in any of the patients. Histologically, the tumors were characterized by an intimate admixture of squamoid/epidermoid cells and mucocytes. The tumors were delineated but not encapsulated with prominent cyst formation and a variable amount of associated fibrosis. The squamoid component showed horny pearl formation, individual cell keratinization and/or the presence of intercellular bridges. Intracytoplasmic and luminal epithelial mucin was observed in all cases. In three of the cases a prominent eosinophilic cellular infiltrate was intimately identified within the neoplastic proliferation. One other case was noteworthy for the presence of ciliated epithelium. In all of the cases the uninvolved thyroid tissue showed the presence of lymphocytic thyroiditis. Rare foci of squamous metaplasia were observed in two of the cases. A microscopic focus of thyroid papillary carcinoma was observed adjacent to the mucoepidermoid carcinoma in one case. Immunohistochemical evaluation of the mucoepidermoid carcinoma showed the following antigenic profile: cytokeratin (five of five), CAM 5.2 (four of four), thyroglobulin (five of six), calcitonin (none of six), chromogranin (none of six), polyclonal carcinoembryonic antigen (four of four), and monoclonal carcinoembryonic antigen (none of five). Surgical excision was the treatment of choice. All of the patients reported are alive without disease (recurrence or metastasis) over periods ranging from 1 to 15 years. Based on our findings, we believe that these tumors are of low-grade malignant potential and originate from thyroid follicular epithelial cells rather than from solid cell nests of the ultimobranchial body.

Adult↗
PubMed1995

Oxyphilic papillary thyroid carcinomas.

Oxyphilic papillary carcinomas of the thyroid have not been extensively studied because they are rare. The morphology and behavior of 34 cases were described. The average age was 44.1 years, the female-to-male ratio was 3.9:1, and the average diameter of the tumors was 2.3 cm. All had papillary structures present. In 31 cases, there was capsular or parenchymal invasion. Six cancers had local lymph node metastases. The average follow-up for 29 patients was 8.1 years. Tumors reappeared in four patients; one patient died from recurrent disease, one patient with disease died due to an unrelated carcinoma, and two patients were treated successfully. Twenty-seven patients at the end of follow-up were alive with no detectable thyroid cancer. The majority of patients remained free of tumor, especially those younger than 50 years.

Adenoma, Oxyphilic↗
PubMed1995

Amyloid goiter. A clinicopathologic study of 14 cases and review of the literature.

The authors report the clinicopathologic features of 14 cases of amyloid goiter (AG). Eleven patients were males and three females with ages ranging from 23 to 75 years (median, 54 years). Eight patients had secondary amyloidosis and six had primary amyloidosis. Nine cases were identified at autopsy. In symptomatic patients (n = 5), the clinical presentation included a nontender, rapidly enlarging neck mass with associated dysphagia, dyspnea or hoarseness. Clinical or laboratory evaluation failed to detect evidence of thyroid dysfunction. The histologic appearance of the thyroid predominantly consisted of diffuse amyloid deposition surrounding thyroid follicles. In two cases, a nodular pattern of amyloid deposition was seen resulting in compression and distortion of the follicular architecture. Areas of mature adipose tissue and focal lymphocytic thyroiditis with or without foreign-body type-giant cells were seen in approximately one third of the cases. Confirmation of amyloid was made by the presence of congophilia and apple-green birefringence under polarized-light microscopy. Immunohistochemical evaluation demonstrated the presence of amyloid A immunoreactivity. No Immunoreactivity was seen with calcitonin or thyroglobulin. Fine-needle aspiration may facilitate the diagnosis, as occurred in one the patients. In symptomatic patients, thyroidectomy is warranted to alleviate pressure symptoms.

Adult↗
PubMed1995

Pancreatoblastoma. A clinicopathologic study and review of the literature.

Pancreatoblastoma is a rare pancreatic tumor with a distinctive histologic appearance that generally affects infants and young children. We have studied 14 cases of pancreatoblastoma and reviewed 41 previously reported examples. Nine of our cases occurred in children (from newborn to 4 years old; mean, 2.4), and five affected adults (from 19 to 56 years old; mean, 40). There were 8 male cases and 6 female cases. Most patients presented with incidental abdominal masses, although pain, weight loss, and obstructive jaundice were present, but rarely. The tumors were very cellular microscopically, with cytologically uniform epithelial cells arranged in sheets and nests. Well-formed acinar structures were a consistent feature, and several cases contained ectatic ductular formations, rarely exhibiting intracellular mucin. Consistently present were squamoid corpuscles: circumscribed, whorled nests of plump spindle cells with a squamous appearance and occasional keratinization. The stroma was moderate to abundant and frequently quite cellular (especially in the pediatric cases). By immunohistochemistry, the tumors exhibited acinar, endocrine, and ductal differentiation, with positivity for pancreatic enzymes (100%), endocrine markers (82%), and carcinoembryonic antigen (85%). Ultrastructural examination most commonly revealed acinar differentiation, although mucigen granules and neurosecretory granules were also occasionally found. The behavior was variable: 36% of patients developed metastases, especially to the liver. The adult patients did poorly: three of five died of tumor (mean survival, 18 months), and two were alive at 5 and 15 months, respectively. In contrast, five of the six evaluable pediatric patients were alive from 22 months to 22 years after diagnosis, and only one died of tumor after 16 months. Good responses to chemotherapy were noted in the pediatric group.

Adult↗
PubMed1994

Only wild-type c-Ki-ras codons 12, 13, and 61 in human pancreatic acinar cell carcinomas.

Activation of the c-Ki-ras proto-oncogene is more common in carcinomas of the pancreas than in other human carcinomas. Most such carcinomas are of the ductal cell phenotype. Ductal adenocarcinomas of the hamster pancreas have similar mutations, but acinar cell carcinomas of the mouse and rat pancreas lack the common c-Ki-ras mutations. Therefore, we examined 11 acinar cell carcinomas of the human pancreas for evidence of mutations at codons 12, 13, and 61. DNA was isolated from tumor cells in paraffin-embedded sections. The polymerase chain reaction was used to amplify the appropriate DNA sequence, and then allele-specific oligonucleotide hybridization and DNA sequence analysis were used to evaluate c-Ki-ras structure. Only wild-type sequences were found in codons 12, 13, and 61. Thus, in both the human and rodent species, mutations of c-Ki-ras appear to be more important in the genesis of ductal carcinomas than in the genesis of acinar cell carcinomas of the pancreas.

Alleles↗
PubMed1994

Mixed acinar-endocrine carcinomas of the pancreas.

Acinar cell carcinoma is a rare pancreatic neoplasm that may contain scattered endocrine cells in as many as 40% of cases. In addition, unusual tumors exist in which the acinar and endocrine components each constitute a significant proportion (> 25%) of the neoplasm; we propose to designate them as "mixed acinar-endocrine carcinomas." In a study of five such cases, we found one case with segregated areas of acinar and endocrine cells that were identifiable in routinely stained sections and four cases with morphologically uniform cell populations where the divergent differentiation was only detected immunohistochemically. The tumors occurred in adults (age range, 48-81; mean, 68); there were two men and three women. None of the patients presented with symptoms related to either enzyme or hormone liberation. Histologically, the tumors were very cellular; various combinations of solid, trabecular, acinar, and glandular growth patterns were noted. The cells contained d-PAS-positive granules and showed immunohistochemical positivity for pancreatic enzymes (trypsin, chymotrypsin, and lipase) and endocrine markers (chromogranin and synaptophysin); specific endocrine hormones were found in two cases. Double immunohistochemical staining for acinar and endocrine markers showed that most cells expressed only one line of differentiation. Ultrastructural study of two cases showed two populations of granules. Two of the patients died of their tumors (mean survival, 10.5 months), one with widespread metastases. Two patients were alive with disease at 12 months after diagnosis, and one patient was lost to follow-up after 3 months. This rare type of pancreatic neoplasm provides further evidence of the close histogenetic relationship between the exocrine and endocrine components of this organ.

Adenoma, Islet Cell↗
PubMed1994

Epithelioid angiosarcoma of the adrenal glands. A clinicopathologic study of nine cases with a discussion of the implications of finding "epithelial-specific" markers.

Adrenal epithelioid angiosarcomas (AEA) are rare neoplasms. We report the clinicopathologic features of nine cases of AEA. AEA occurred most frequently in the sixth and seventh decades of life (age range, 45-85 years; median, 60); five cases occurred in men and four in women. Presenting symptoms included abdominal mass with or without pain, weight loss, fever, and weakness. Two cases were asymptomatic; one was discovered during evaluation for other disease(s) and the other at autopsy. All neoplasms were nonfunctioning. Radiographic evaluation demonstrated suprarenal or retroperitoneal neoplasms ranging in size from 6 to 10 cm in greatest dimension. Histologically, the neoplasms were invasive, predominantly arranged in solid sheets or nests, and composed of epithelioid cells. Endothelial cell differentiation was suggested by the transition areas between dilated anastomotic vascular spaces and the sheet-like growth, the cytomorphologic similarity between the endothelial cells lining the discernible vascular spaces and those seen in the solid foci, and the presence of intracytoplasmic vacuolization occasionally containing red blood cells. Endothelial derivation was confirmed by immunohistochemistry including Factor VIII-related antigen (FVIII), CD-34 (hematopoetic progenitor cell antigen), and/or Ulex europaeus agglutinin-1 lectin immunoreactivity (UEA-1) and by ultrastructural findings, including rod-shaped microtubulated bodies and intracytoplasmic lumen formation. In addition, cytokeratin reactivity was seen in seven cases, and B72.3 (tumor-associated glycoprotein-72) reactivity was seen in six. Surgical resection was the treatment of choice, occasionally supplemented by chemotherapy. Three patients are presently alive, free of disease, at 13, 11, and 6 years following diagnosis. Three died with metastatic AEA of the lung, and three died of unrelated causes.

Adrenal Gland Neoplasms↗
PubMed1992

Acinar cell carcinoma of the pancreas. A clinicopathologic study of 28 cases.

We have examined the microscopic appearance, immunohistochemical staining properties, and clinical behavior of 28 cases of acinar cell carcinoma of the pancreas. Two of the tumors occurred in children. The adult patients ranged in age from 40 to 81 years (mean, 62 years). Males greatly outnumbered females, and most of the patients were white. Presenting symptoms were nonspecific, and jaundice was infrequent. The frequently reported complications from increased serum lipase levels (i.e., arthralgias and subcutaneous fat necrosis) were present in only 16% of the patients. Grossly, the tumors were relatively circumscribed and fleshy, averaging 10.8 cm, with occasionally extensive hemorrhage and necrosis. Microscopically, the tumors were very cellular and characteristically lacked a desmoplastic stroma. Acinar, solid, trabecular, and glandular patterns of growth were identified; individual tumors were usually mixed. Nuclei were round to oval, with minimal pleomorphism and single prominent nucleoli. Mitotic activity was variable. In general the cytoplasm was moderately abundant, eosinophilic, and granular, but many of the solid tumors had cells with scanty cytoplasm. Characteristic periodic acid-Schiff-positive, diastase-resistant cytoplasmic granules were demonstrated in greater than 90% of the cases, and the butyrate esterase histochemical stain for lipase activity was positive in 73%. Immunohistochemically, there was positivity for trypsin in 100% of the cases, for lipase in 77%, for chymotrypsin in 38%, and for amylase in 31%. A minor endocrine component was recognized with antibodies against chromogranin or islet cell hormones in 42% of the tumors. Ultrastructurally, exocrine secretory features were present, with polarized cells showing microvillilined lumina, abundant rough endoplasmic reticulum, and 125-1,000-nm zymogen-like granules. In addition, many cases showed pleomorphic electron-dense granules measuring up to 3,500 nm and containing fibrillary internal structures. Follow-up information was available in 88% of the cases. Half of the patients had metastatic disease at presentation and an additional 23% subsequently developed metastases, which were usually restricted to the regional lymph nodes and liver. The mean survival for all cases was 18 months, with 1- and 3-year survivals of 57 and 26%, respectively. Patients presenting before age 60 years survived nearly twice as long as older patients did. Stage also influenced prognosis, whereas the histologic subtype of the tumors and the location within the pancreas correlated only weakly with survival.

Adult↗
PubMed1989

Fat-containing lesions of the thyroid gland.

Thyroid lesions containing adipose tissue are rare; only scattered case reports are recorded. This study of 17 lipomatous thyroid lesions on file at the Armed Forces Institute of Pathology was undertaken to better document the full range of these abnormalities. Patient ages ranged from 11 to 75 years; nine patients were female and eight were male. Fat-containing thyroid neoplasms included seven papillary carcinomas, four adenomatoid nodules, one follicular adenoma, and one minimally invasive follicular carcinoma. Nonneoplastic conditions associated with adipose tissue included four cases of amyloid goiter, two cases of lymphocytic thyroiditis, one case of dyshormonogenetic goiter, and one case of thyroid atrophy. This study documents the presence of adipose tissue in a wider range of benign and malignant thyroid lesions than has previously been reported.

Adipose Tissue↗