Elson B. Helwig

Angiolymphoid hyperplasia with eosinophilia: A clinicopathologic study of 116 patients

The clinicopathologic spectrum of angiolymphoid hyperplasia with eosinophilia (ALHE) is reported through a study of 116 cases (67 male and 49 female) submitted to the Armed Forces Institute of Pathology. The lesions are most common in the head-neck region and are characterized by single or multiple smooth-top papules or plaques of varying color. Microscopically, ALHE consists of anomalous vascular proliferations and varying degrees of nodular and diffuse lymphocytic infiltrates with eosinophils at all levels of the corium and subcutaneous tissue. In fifty-three cases an arterial structure, confirmed by the presence of an internal elastic lamina, was observed in close association with venular structures or was the site of endothelial cell proliferation. ALHE comprises a spectrum of unusual vascular proliferation with inflammation, encompassing such entities as inflammatory angiomatous nodules, pseudo or atypical pyogenic granuloma, histiocytoid hemangioma, epithelioid hemangioma, and Kimuras disease. The existence of arterial structures among venules and endothelial cell proliferations suggests the presence of arteriovenous (AV) shunts, which may help explain the pathogenesis and biologic behavior of this condition.

Atypical fibroxanthoma of the skin. A clinicopathologic study of 140 cases

In an attempt to further understand the nature of atypical fibroxanthoma of the skin, 140 lesions were subjected to clinical, histologic, and histochemical studies. Atypical fibroxanthoma most commonly presented as a solitary, nonspecific nodule or ulceronodule on exposed skin of the face in the elderly. A clinical variant occurred in much younger persons on the covered areas of the trunk and limbs. Histologically, atypical fibroxanthoma develops as a circumscribed, cellular proliferation within the dermis, with occasional infiltration into the subcutis. Morphological patterns vary from lesions showing plump spindle cells in interlacing fascicles to those with haphazardly arranged large polyhedral cells. Bizarre multinucleated giant cells with foamy cytoplasm and numerous mitotic figures enhance its distrubing sarcoma‐like appearance. Among 101 patients followed for periods up to 15 years, no metastatic lesions were found and only nine lesions recurred. Correlation of the clinical and follow‐up data support the concept that in spite of its alarming histologic appearance, atypical fibroxanthoma of skin appears to behave in a benign manner.

Gastric epithelioid leiomyoma and leiomyosarcoma (leiomyoblastoma).

A series of 127 surgical specimens of epithelioid leiomyomatous tumors (leiomyoblastomas) of the gastric wall from the files of the Armed Forces Institute of Pathology (AFIP) were studied as to biologic behavior, morphogenesis, and histologic features of value in distinguishing benign and malignant variants. These tumors affect middle‐aged men primarily and usually present with upper gastrointestinal bleeding or peptic ulcer‐like symptoms. They are composed of a mixture of round epithelioid and spindle cells, many of which have clear cytoplasm. The cells are ensheathed by delicate reticular fibers. The presence of a perithelial or glomoid pattern in some tumors suggests a possible relationship to angiomyoma, glomus tumors, and “pericytoma” The epithelioid leiomyoma, the benign form, often arises in the mid‐ and distal stomach, especially on the anterior wall. Microscopically, it is recognized by the presence of large epithelioid cells and infrequent mitotic figures. Of 103 epithelioid leiomyomas, only one metastasized and thus was biologically malignant. The epithelioid leiomyosarcoma often arises in the proximal stomach and also distally, especially on the posterior wall. Two histologic types of epithelioid leiomyosarcoma are distinguished from the benign epithelioid leiomyoma by the small size of the cells and occasional higher mitotic counts. One sarcoma variant is a small cell caricature of the leiomyoma. The other is more anaplastic, associated with a loss of reticular fibers surrounding the cells and an alveolar arrangement. Epithelioid leiomyosarcomas are the most common type of gastric sarcoma. They are aggressive neoplasms; 63% metastasized, usually within 2 years after diagnosis.

Cutaneous sebaceous neoplasms

Ninety‐five patients, each having a single cutaneous sebaceous neoplasm, were selected for study. Lesions of the eyelids were excluded. The tumors were classified as sebaceous adenomas (46), basal cell carcinomas with sebaceous differentiation (43), and sebaceous carcinomas (6). Morphological criteria for separation into these categories are discussed and illustrated. Lesions considered transitional between the classes were observed. Recurrence was unusual, occurring in only three sebaceous adenomas, three basal cell carcinomas with sebaceous differentiation, and one sebaceous carcinoma. Only one tumor, a sebaceous carcinoma, showed aggressive local growth. None metastasized. This behavior contrasts with the frequently observed metastases and death associated with sebaceous carcinoma arising in eyelids, caruncle, or orbit.

Metastatic basal cell carcinoma: A clinicopathologic study of seventeen cases

Basal cell carcinoma is a common cutaneous neoplasm that rarely metastasizes. We studied the clinical and pathologic features of 17 patients with metastatic basal cell carcinoma as recorded in the files of the Armed Forces Institute of Pathology (AFIP). Sixteen of the patients were male, and as far as it could be determined, all were white. The most frequent site of metastasis was lung (9 cases), followed by bone (5), lymph nodes (4), liver (3), spleen (1), and adrenal gland (1). Thirteen of the patients had metastatic lesions involving only one organ system. Mean survival time after metastasis was 1.6 years. Features of metatypical (basosquamous) basal cell carcinoma were common in the primary and recurrent tumors, and metastatic lesions generally had a metatypical or adenoid pattern. Two of the five bony metastases demonstrated shadow cells characteristic of pilomatrixoma. The metatypical pattern of a basal cell carcinoma is a feature of an aggressive lesion with the ability to metastasize.

Adenocarcinoid, a mucin-producing carcinoid tumor of the appendix: a study of 39 cases.

Adenocarcinoid is a form of appendiceal carcinoid possessing features of both carcinoid and adenocarcinoma. There are two histologic types. Thirty patients had the goblet cell type, characterized by nests of large mucin‐distended cells. Nine patients had the tubular type, characterized by small glandular structures lined by uniform cells. Despite abundant mucin and a goblet cell or acinar‐like arrangement, a closer relationship to carcinoid than to adenocarcinoma is suggested by a concentration of tumor elements below the crypts of Lieberkuhn, a lack of evidence of neoplastic transformation of the appendiceal mucosa, and the demonstration of argentaffin or argyrophil granules in 88% of the lesions. Six tumors, all of the goblet cell type, metastasized and resulted in the death of the patients. One of the tumors that metastasized had a prominent tubular component. Most adenocarcinoids can be adequately treated by appendectomy, but hemicolectomy is recommended for those tumors showing atypical foci, a high mitotic count, or spread beyond the appendix. Cancer 42:2781–2793, 1978.

Cutaneous meningiomas—a clinicopathologic study

Twenty‐five cases of cutaneous meningioma and related processes were reviewed. Three types of meningioma cutis were noted. Type I occurs in the scalp, face, or paravertebral region of children and young adults, is usually present at birth, and generally has a benign course. This primary cutaneous meningioma bears similarities to developmental defects and probably originates from arachnoid cell rests displaced during embryogenesis into the cutis or subcutis. Type II occurs around sensory organs of the head and along the course of cranial and spinal nerves. It generally appears in adults as a de novo lesion and represents a cutaneous extension from an ectopic soft tissue meningioma, probably arising from arachnoid cell rests displaced along nerve sheaths. Type III represents an extension into the skin from a central nervous system meningioma infiltrating across bone or a bone defect. Prognosis for Types II and III is less favorable than for Type I. Clinically, cutaneous meningiomas resemble a variety of common skin lesions. The histopathologic appearance is usually but not always characteristic.

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 ofte, 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.

Atypical fibroxanthoma of the skin with metastasis

Cutaneous atypical fibroxanthoma (AFX) occurs in elderly persons as a small nodule or ulceronodule in actinically damaged skin of the head and neck area. The vast majority of AFX behave in a benign manner, and metastasis is rare. Eight examples of metastasizing AFX are reported. Factors that portend agressive behavior and metastasis are vascular invasion, recurrence, deep tissue invasion, tumor necrosis and, possibly, defective or depressed host resistance. The metastasizing primary AFX were located on the head, and the metastasis involved the structures in the region of the parotid gland.

Cutaneous lymphoid hyperplasia.

Observations on 193 patients with 225 biopsied lesions showing heavy lymphoid infiltrates in the skin are presented. Lesions of cutaneous lymphoid hyperplasia are compared with those of cutaneous malignant lymphoma, and histopathologic criteria for their separation are discussed. The benign lesions could usually be separated from the malignant ones by a higher incidence of epidermal, stromal, and vascular abnormalities and by the presence of a polymorphous cellular infiltrate of well‐differentiated cells. Lesions of cutaneous lymphoid hyperplasia fall into lymphoreticular, granulomatous, and follicular histopathologic patterns, depending on the nature of the infiltrate. The malignant potential of cutaneous lymphoid hyperplasia is low, but rare examples of the association of the malignant with the benign have been recorded.