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Biomedical subjects

E B Helwig

Publications and source records attributed to E B Helwig.

At least 19 recordsLinked to original sources

Proliferating epithelial cysts. Clinicopathological analysis of 96 cases.

Ninety-six proliferating cutaneous epithelial cysts were classified into two subtypes, proliferating trichilemmal cysts (PTC) and proliferating epidermoid cysts (PEC), depending on the mode of keratinization or the origin of the tumors. The clinicopathological features and the biological behavior of these two subtypes were compared. Among 63 patients with PTC, 45 (71%) were women and 18 (29%) were men. The most common site was the scalp (78%), followed by the trunk (13%). These tumors were well circumscribed subepidermal lesions and demonstrated uniform histologic pattern with varying degrees of cytologic atypia. A few tumors extended into the epidermis and occasionally became ulcerated. Follow-up of 59 (94%) PTC for an average of 4 years revealed recurrence in one. Ten tumors demonstrated carcinomatous changes including one with anaplastic carcinoma and regional lymph node metastasis. None of these tumors recurred or developed further metastasis following wide excision. Of 33 PECs, 12 (36%) occurred in women and 21 (64%) in men. These tumors were widely distributed in the pelvic and anogenital areas (36%), followed by the scalp (21%), upper extremities (18%), and trunk (15%). Seventy-nine percent of the PECs were located in areas outside the scalp. The PECs were subepidermal tumors but often communicated to the surface. The histologic pattern of PEC was more variable than that of PTC. Seven tumors exhibited carcinomatous changes. Follow-up of 30 (91%) PEC revealed local recurrences in 6, with multiple recurrences in 3, and extensive local invasion in 2, resulting in death in one. Greater anaplasia, high mitotic rate and deeper invasion were associated with increased incidence of recurrence and aggressive behavior. Although both PTC and PEC were locally aggressive tumors and potentially malignant, distant metastasis was unusual. These tumors should be treated with wide local excision, especially those showing cytologic atypia and carcinomatous changes.

Adult↗

Pigmented spindle cell nevus: a clinicopathologic analysis of ninety-five cases.

BACKGROUND: Pigmented spindle cell nevus (PSCN) is often interpreted as a Spitz nevus or misdiagnosed as malignant melanoma. OBJECTIVE: The purpose of this study was to analyze the clinical and histologic features and to determine the biologic behavior of 95 cases of PSCN. METHODS: We reviewed clinical data, follow-up information, and microscopic features of all 95 cases of PSCN. RESULTS: PSCNs are dark brown to black, 3 to 6 mm in diameter, and occur most commonly on the extremities (75%) and back (16%) with a predilection for the legs. These lesions are more common in women in the third decade of life. Microscopically, PSCNs are characterized by uniform, spindle-shaped, pigmented melanocytes. Although some histologic features overlap with those in spindle and epithelioid cell nevus, PSCN is a separate entity. In addition, PSCN must be differentiated from malignant melanoma. Fifty-seven patients (60%) observed for an average of 6 years did not develop local recurrence or metastasis. CONCLUSION: PSCN is a distinctive, acquired, benign melanocytic lesion, that should not be confused with spindle and epithelioid cell nevus or malignant melanoma. Complete excision is recommended for treatment.

Adolescent↗

Balloon cell malignant melanoma of the skin. A clinicopathologic study of 34 cases with histochemical, immunohistochemical, and ultrastructural observations.

Balloon cell malignant melanoma (BCMM) is a rare histologic variant of malignant melanoma (MM). Thirty-four patients with BCMM from the files of the Armed Forces Institute of Pathology (AFIP) were studied by means of clinicopathologic correlation and histochemical, immunohistochemical, and ultrastructural methods to better define this entity. The cytoplasmic features of the balloon cells observed in BCMM resemble those noticed in balloon cell nevus (BCN), but the presence of nuclear pleomorphism, atypia, and mitoses and the absence of intervening stroma help distinguish BCMM. The cells also show many histochemical, immunochemical, and ultrastructural features of conventional melanoma cells. Although it is generally believed that balloon melanoma cells represent a degenerative change, the immunohistochemical and electron microscopic findings suggest that the balloon tumor cells are most likely metabolically active melanocytic cells. Microscopically, BCMM also must be differentiated from other clear cell tumors such as clear cell sarcoma (MM of soft parts), hibernoma, xanthoma, sebaceous neoplasms, metastatic renal cell carcinoma, (malignant) clear cell acrospiroma, (malignant) granular cell tumor, granular (clear) cell basal cell carcinoma, clear cell syringoma, and atypical fibroxanthoma. The prognosis of BCMM usually correlates with the tumor thickness similar to that in other histologic types of cutaneous MM. Nineteen (57.5%) of 33 patients with adequate follow-up information died of disseminated tumors from 2 months to 12 years after the initial treatment. Six (18.2%) patients developed local recurrences: four of these patients died of metastasis and two were alive with metastatic tumor at last contact. Five (15.2%) patients were alive with metastatic tumors, and seven (21.2%) were alive without evidence of disease at last contact. Recognition of BCMM is important because of its malignant biologic behavior.

Adult↗

Deceptive bizarre stromal cells in polyps and ulcers of the gastrointestinal tract.

The clinical and pathologic features of 33 pseudomalignant lesions of the gastrointestinal tract with bizarre stromal cells are reported. Deceptive histologic changes were identified in ulcers of seven patients and in inflammatory polyps of 26. A misdiagnosis of malignant neoplasm was made in six of the 33 patients (three polyps and three ulcers). A history of gastrointestinal bleeding and/or inflammatory bowel disease was common. The bizarre stromal cells were usually distributed beneath the ulcerated mucosa or within granulation tissue. They stained strongly for vimentin in 20 of 23 cases. Some of the bizarre cells also stained for muscle specific actin (seven of 23 cases). The cells appear to be reactive fibroblasts or myofibroblasts. Follow-up information obtained on 24 of the 33 patients (including four of the six cases initially diagnosed as malignant) revealed 22 patients to be alive without evidence of a malignant neoplasm (average follow-up, 13 months). Two patients died of other causes. Correct recognition of these bizarre stromal cells in gastrointestinal ulcers and inflammatory polyps will prevent a potentially serious diagnostic pitfall.

Adult↗

Dysplasia of the stomach and Barrett esophagus: a follow-up study.

Seventy-seven patients with biopsy-proven glandular dysplasia of the esophagus (39 patients) or stomach (38 patients) were followed for a mean of 44 mo or until resection. Of 34 patients with low-grade dysplasia at initial biopsy, 29 had no evidence of high-grade dysplasia or carcinoma on follow-up, three died of other causes, and two had severe dysplasia diagnosed on subsequent biopsy. Of 43 patients with high-grade dysplasia, 28 had no evidence of dysplasia on follow-up (four died of unrelated causes and two of postoperative complications). Fifteen were shown to have invasive carcinoma. Thirteen of the 15 diagnoses of invasive carcinoma were made within 12 mo of original endoscopy. Ulcerated high-grade dysplasias were more likely associated with carcinoma than were nonulcerated high-grade dysplasias (P = 0.001). The presence of ulceration and short time interval to the progression of carcinoma suggest that carcinoma may have been present at the time of initial biopsy in many cases. The frequency of adenocarcinoma's arising in severe dysplasia was slightly less in the stomach (29%) than in the esophagus (41%). We conclude that low-grade dysplasia is often indolent and that ulcerated high-grade dysplasia is often a marker for adjacent invasion.

Adult↗

Somatostatin-producing duodenal carcinoids in patients with von Recklinghausen's neurofibromatosis. A predilection for black patients.

Eight patients with von Recklinghausen's disease (VRD) and duodenal carcinoids are presented. Seven patients were black, and one white. Six of the eight were women. The presenting symptom was either jaundice or abdominal pain. All tumors were located in the second portion of the duodenum, and three were multiple. Associated tumors other than neurofibromas included multiple leiomyomas, meningioma, neurofibrosarcoma, and prostatic sarcoma. Seven tumors had psammoma bodies, and in three they were numerous. Somatostatin-positive cells were demonstrated in all cases. Two tumors had spread to regional lymph nodes at the time of surgery. There appears to be a predilection for black patients among those with VRD and duodenal carcinoids.

Adult↗

Goblet cell carcinoids and related tumors of the vermiform appendix.

Appendiceal carcinoids with glandular differentiation pose difficulties in classification and prediction of clinical behavior. Sixty-four such cases were divided into three histologic groups on the basis of routine and immunohistochemical stains: (1) Tubular carcinoids were small and confined to the appendix, had small amounts of intraluminal mucin with few or no goblet cells, were nonargentaffin, lacked serotonin, and were diffusely positive for glucagon. All ten with follow-up (mean, 17 months) were without metastasis. (2) Goblet cell carcinoids were confined to the appendix and mesoappendix, circumferentially surrounded the appendiceal lumen, and were often not suspected grossly. Histologically, they were often mixed with small crypt-like glands and were serotonin positive. All 22 with follow-up (mean, 19 months) were without metastasis whether or not right hemicolectomy was performed. (3) Mixed carcinoid-adenocarcinomas showed spread into the cecum or adjacent viscera at the time of diagnosis and had a large carcinomatous pattern with areas of mucinous, signet-ring, or single-file structure, in addition to goblet cell or insular carcinoid. All patients had right hemicolectomies, and all but two with follow-up died of the disease (mean, 16 months). Although a histologic spectrum exists among carcinoid tumors and certain adenocarcinomas of the appendix, it is possible to delineate three biologically distinct groups. Surgical margins should be taken of all appendices because these tumors often do not form discrete masses.

Adenocarcinoma↗

Carcinoid tumors of the duodenum. A clinicopathologic study of 99 cases.

Ninety-nine carcinoid tumors of the duodenum were studied. Seventy-seven patients were followed up for a mean period of 65 months, 20 tumors were autopsy findings, and two patients were unavailable for follow-up. Sixteen tumors (21%) produced metastases, all discovered initially; 3 patients (4%) died from metastatic disease (mean survival, 37 months postoperatively). Features associated with metastatic risk were involvement of muscularis propria, size greater than 2 cm, and the presence of mitotic figures. For 51 tumors, there was no correlation between immunohistochemical somatostatin and history of diarrhea, cholelithiasis, or diabetes mellitus (somatostatin syndrome). Five tumors were associated with Zollinger-Ellison syndrome and had immunohistochemical gastrin, but in the others there was no correlation between ulcer disease and gastrin positivity. Duodenal carcinoids are indolent, especially when small and localized to the submucosa. Immunohistochemical identification of somatostatin and gastrin has little clinical relevance.

Adult↗

Intra-abdominal fibromatosis. A pathologic analysis of 130 tumors with comparison of clinical subgroups.

We studied the clinical, gross, and histologic findings of 130 fibromatoses of the mesentery and other peritoneal sites. Seventeen patients had Gardner syndrome, 12 had prior abdominal surgery, and six had apparent estrogen elevation, including five pregnant or postpartum women and an alcoholic male with gynecomastia. The tumors were usually large and grossly circumscribed. Most often, they were located in the mesentery of the small bowel. They were multiple in 18 cases. Typical histologic features included a dense, collagenous stroma; prominent, dilated, thin-walled vessels; muscular hyperplasia of small arteries; keloidal change; myxoid change; and fibrous tissue insinuation into the muscularis propria of the bowel. Although mitoses were noted in many tumors, they were usually few in number. The gross and histologic features were similar in the clinical subgroups; however, keloidal change was seen less often in female patients. Less than half of the cases were initially correctly diagnosed. Most patients without Gardner syndrome were without recurrence at follow-up, even when the lesions had been incompletely excised.

Abdomen↗

Gangliocytic paraganglioma.

A study of gangliocytic paragangliomas (GPs) of the gastrointestinal tract from 51 patients showed characteristic microscopic features: epithelioid cells with an endocrine growth pattern, spindle cells, and ganglion cells. Forty-nine tumors were located in the duodenum, 1 in the jejunum, and 1 in the pylorus. Twenty-one patients were female, 28 male, and for two the sex was unknown. The average age at presentation was 54 years (range, 23-83). No patient had a recurrence. No neuroendocrine syndrome was found in any patient or patient's family. Immunohistochemical stains in 33 cases yielded the following (proportion positive): S-100 protein 94%, synaptophysin 94%, neuron-specific enolase 94%, pancreatic polypeptide 88%, somatostatin 75%, chromogranin 72%, neurofilament protein 64%, keratin 52%, leu-enkephalin 48%, serotonin (one case), and gastrin (one case). Antisera usually stained one or two of the three major cell types. Pancreatic-type tissue was identified in or near 28 tumors, including the pyloric and jejunal lesions and two in the distal duodenum. The authors conclude that GP is benign; is not associated with endocrine syndromes; contains autonomic, neural, and endocrine cell types; and is related to pancreatic development.

Adult↗

Deep penetrating nevus.

We report a clinical and histologic study of 70 patients, each with a single melanocytic lesion termed "deep penetrating nevus" (DPN). The lesions are most commonly found on the face, upper trunk, or proximal extremities of patients between the ages of 10 and 30 years. Typically they are darkly pigmented. Histologically they are characterized by loosely organized nests of pleomorphic pigmented cells that penetrate deep into the reticular dermis and often to the subcutaneous fat. Follow-up was obtained from 48 patients. It ranged from 1 to 23 years (mean, 7 years). Despite an initial histologic diagnosis of malignant melanoma in 29% of the cases, there were no local recurrences and no distant metastases. It is important to differentiate DPN from malignant melanoma. The characteristic histologic features of DPN also allow its differentiation from spindle cell and epithelioid cell nevi and blue nevi.

Adolescent↗

Carcinoids of the duodenum. A histologic and immunohistochemical study of 65 tumors.

The light-microscopic and immunohistochemical characteristics of 65 duodenal carcinoids are presented. Most tumors showed a mixture of cribriform, insular, glandular, solid, and trabecular growth patterns. Eighty-five percent of the tumors were argyrophil and 15% argentaffin. The nonspecific neuroendocrine markers chromogranin, Leu-7, and neuron-specific enolase were positive in 97, 91, and 83% of tumors, respectively. Immunoreactivity for specific hormones/amines were as follows (percent positive tumors): somatostatin, 47%; N-gastrin, 56%; serotonin, 39%; calcitonin, 19%; insulin, 5%; pancreatic polypeptide, 3%; adrenal corticotropic hormone, 0%; glucagon, 0%. Sixty-eight percent had gastrin/cholecystokinin-like reactivity. Ten psammomatous tumors were located near the ampulla; eight were somatostatin positive, including two in patients with neurofibromatosis. One additional tumor in a patient with neurofibromatosis lacked psammoma bodies but elaborated somatostatin. Eight additional tumors in nonneurofibromatosis patients produced solely somatostatin. Duodenal carcinoids often elaborate more than one polypeptide hormone; those in the ampulla often elaborate somatostatin and have psammoma bodies.

Antigens, Differentiation↗

Tailgut cysts. Report of 53 cases.

Fifty-three examples of developmental "tailgut cysts" in the retrorectal space occurred predominantly in women and caused symptoms of mass effect or pain in 51%. The lesions were usually multicystic and lined by a variety of epithelial types, including ciliated columnar, mucin-secreting columnar, transitional, and squamous epithelium. Inflammation was present in 50%. In one patient a poorly differentiated adenocarcinoma was present. These cysts are most likely derived from remnants of the embryonic tailgut and differ from teratomas. Complete excision of the multilocular and multicystic process prevents recurrent draining sinuses and eliminates the possibility of malignant change.

Adolescent↗

Stromal tumors of the gastrointestinal tract: myogenic or neurogenic?

Because of the recent controversy over the histogenesis of gastrointestinal stromal tumors, 170 gastrointestinal tract tumors appearing to be of smooth muscle type with routine stains were studied immunohistochemically. Five percent were positive for S-100 ("neural") protein. These findings are compared with several recent studies. We conclude that only a small minority of gastrointestinal tract stromal tumors are neurogenic and that most are probably of smooth muscle type.

Adolescent↗

Aggressive digital papillary adenoma and adenocarcinoma. A clinicopathological study of 57 patients, with histochemical, immunopathological, and ultrastructural observations.

Fifty-seven examples of a rare eccrine sweat gland tumor (aggressive digital papillary adenoma and adenocarcinoma) were studied by means of light microscopy, electron microscopy, and immunoperoxidase techniques. The neoplasm occurred as a single, painless mass, almost exclusively on the fingers, toes, and adjacent skin of the palms and soles. Microscopic features were distinct from those of other eccrine sweat gland tumors and often led to the diagnosis of such metastatic carcinoma as that of the breast. The characteristic histologic features included tubuloalveolar and ductal structures with areas of papillary projections protruding into cystic lumina. The stroma varied from thin, fibrous septae to areas of dense, hyalinized collagen. Forty tumors were classified as adenoma (ADPA) and 17 as adenocarcinoma (ADPAca). Histologically, ADPAca was distinguished from ADPA by its poor glandular differentiation and by necrosis, cellular atypia and pleomorphism, invasion of soft tissue and bone, and invasion of blood vessels. Eighteen (50%) patients with ADPA and 8 (47.0%) who had ADPAca developed recurrent lesions (2 months to 9 years) after surgical removal of the tumor. Seven (41.2%) patients with ADPAca developed metastases, of which 5 involved the lung. Three patients died of metastases, 5 to 20 years after surgical treatment of the primary tumor. The histologic malignant features in ADPAca are indicative of potential for distant metastasis and fatality. The recognition of aggressive digital papillary adenoma and adenocarcinoma as a distinct clinicopathological eccrine sweat gland neoplasm is important because of the potential for aggressive local growth and distant metastasis.

Adenocarcinoma, Papillary↗

Morphometry and cytophotometric assessment of DNA in smooth-muscle tumors (leiomyomas and leiomyosarcomas) of the gastrointestinal tract.

The difficulties in predicting the biologic behavior of gastrointestinal (GI) smooth-muscle tumors (leiomyomas and leiomyosarcomas) based on the usual criteria of malignancy are discussed. In order to evaluate the prognostic importance of the nuclear DNA content and nuclear dimensions, measurements were performed on Feulgen-stained sections of GI smooth-muscle tumors from 66 patients. The best discrimination between benign and malignant tumors was obtained by using DNA index and tumor size as descriptors in a linear discriminate analysis. This method separated 79% of the benign and 97% of the malignant smooth-muscle tumors. However, as with conventional criteria for malignancy, there remained a group of tumors close to the discriminating line with an indeterminate malignant potential. In an attempt to reduce the number of such indeterminate tumors, future studies will include the use of several descriptors in a multivariate analysis system and the application of flow cytometric studies to all tumors.

Adolescent↗

Prostatic acid phosphatase activity in carcinoid tumors.

In a study of 105 gastrointestinal carcinoid tumors, 67% of the rectal carcinoids and 15% of the carcinoids at other gastrointestinal sites were positive, immunohistochemically, for prostatic acid phosphatase activity. These findings are relevant to the differential diagnosis between rectal carcinoids and prostatic carcinoma.

Acid Phosphatase↗

Bowenoid papulosis. A clinicopathologic study with ultrastructural observations.

One hundred eight patients were studied who had anogenital lesions showing microscopic features as seen in bowenoid papulosis (BP), a recently described condition occurring most commonly in young adults. Patients typically show multiple papules, small nodules, or plaques that clinically mimic verrucae or nevocellular nevi. Although the lesions show microscopic cytologic atypia, a distinction from Bowen's disease, erythroplasia of Queyrat, and other forms of carcinoma in situ can usually be made on the basis of histologic and clinical criteria. The disorder responds to conservative treatment, although recurrences are not uncommon. Evolution of the lesions to invasive carcinoma was not observed. Mounting evidence links the development of BP to infection with human papilloma virus, but other viruses, as well as hormonal and immunologic factors, may also play a role.

Adolescent↗