Ramon L. Font

Fibrous histiocytoma of the orbit. A clinicopathologic study of 150 cases.

This is a clinicopathologic study of 150 cases of fibrous histiocytoma of the orbit. The tumors occurred in adults (median age, 43 years). The upper and nasal portions of the orbit were the most common sites of involvement. The most common signs and symptoms were proptosis (60 per cent), mass (46 per cent), and decreased vision (25 per cent). Based on the histopathologic features, the tumors were classified in three groups: benign (94 cases), locally aggressive (39 cases), and malignant (17 cases). The biological behavior correlated well with the duration of symptoms, the size and margins of the mass, and the histologic classification. The rate of recurrence was 31 per cent for the benign tumors, 57 per cent for the locally aggressive tumors, and 64 per cent for the malignant tumors. Follow-up data were obtained for 123 patients, with a mean duration of seven years. The ten-year survival of patients with benign, locally aggressive, and malignant fibrous histiocytoma was 100 per cent, 92 per cent, and 23 per cent, respectively. Nine patients died as a result of the tumor, six from local invasion of adjacent structures and three from metastatic disease. Fibrous histiocytoma is the most common primary mesenchymal orbital tumor of adults. The origin of the neoplasm is probably a primitive mesenchymal cell. Complete surgical excision appears to be the treatment of choice.

Ophthalmologic Manifestations of Granulocytic Sarcoma (Myeloid Sarcoma or Chloroma):The Third Pan American Association of Ophthalmology and American Journal of Ophthalmology Lecture

The clinicopathologic review of 33 well-documented cases of granulocytic sarcoma on file in the Registry of Ophthalmic Pathology confirmed the facts that this tumor is encountered mainly in children, that boys are affected more frequently than girls, and that white Americans appear to be less vulnerable than other ethnic groups. While granulocytic sarcoma is a variant of granulocytic leukemia, the tumor may appear before, after, or concomitantly with hematologic evidence of leukemia. In the present series only four of the 33 patients were already known to have leukemia when they were first seen by an ophthalmologist for their orbital, ocular, or adnexal lessions. The Leder stain has proved extremely helpful in arriving at a definitive histopathologic diagnosis. Accurate diagnosis is important to prevent inappropriate medical or surgical treatment and to indicate the need for vigorous antileukemic chemotherapy. Prognosis at best is poor.

Mucinous sweat gland adenocarcinoma of eyelid. A clinicopathologic study of 21 cases with histochemical and electron microscopic observations

This is a clinicopathologic study of 21 cases of mucinous sweat gland adenocarcinoma involving the eyelid. The tumor occurred in middle‐aged adults (median age 60 years) and has a predilection for males. In this series eight patients (40%) had one or more local recurrences, one of whom died with extensive local invasion of the face after multiple recurrences in a 15‐year interval. Only one patient had metastasis to the submandibular lymph nodes, which was treated by radical neck dissection. Involvement of the head, especially the face and scalp, was observed in 78% of the cases from the literature. Combining our cases with those previously reported, we found that the eyelid was involved in almost half of the cases (21 out of 45 lesions).

Hemangiopericytoma of the orbit: A clinicopathologic study of 30 cases

The clinical and pathologic features of 30 orbital hemangiopericytomas were reviewed. An effort was made to determine the clincopathologic characteristics that may correlate with the biologic behavior. Histopathologic criteria evaluated included degree of cellularity, nuclear atypia, mitotic activity, tumor size, and the presence of hemorrhage and necrosis. Histopathologically, 16 tumors were classified as benign, five as borderline, and nine as malignant. Follow-up information was obtained in 27 cases (mean duration of follow-up period was five and one-half years). Eight tumors recurred (30 per cent). Five patients died, four with widespread metastases (15 per cent) and one of unrelated causes. Tumors that recurred or metastasized were distributed among all three histologic groups. The interval between the onset of symptoms and metastasis for three of the four lethal tumors was 31 years, 28 years, and 8 1/2 years; in the fourth case it was unknown. For the entire series, the five-year actuarial survival rate was 89 per cent. Although the number of cases is too small to serve as a basis for firm conclusions, the lack of unequivocal correlation between the histologic features and clinical behavior is consistent with the unpredictable behavior of this neoplasm, as concluded by other investigators. Because of the apparent circumscription of most of these tumors, complete surgical excision rather than exenteration is recommended. Metastasis usually develops late in the clinical course of the disease, indicating that long-term follow-up studies are required before a cure can be assumed.

Metastatic mammary carcinoma in the eyelid with histiocytoid appearance.

A clinicopathologic study of 13 cases of carcinoma of the breast with metastasis to the eyelids has been made. Seven of these presented diagnostic problems for several reasons: 1. because the lesion in the lid became symptomatic before the primary tumor of the breast was detected; 2. because it was the first sign of metastasis at least 2 years after an apparently curative mastectomy, and/or 3. because of a peculiar histiocytoid pattern of the metastatic tumor that mimicked a benign histiocytic lesion. The histiocytoid pattern, however, once recogognized, is highly suggestive of a metastasis from a primary mammary carcinoma. Of the 13 metastatic tumors in the lid, only 3 had the histopathologic pattern of a typical infiltrating ductal carcinoma. Two had sufficiently pleomorphic patterns that the diagnosis of malignancy was inescapable. Eight tumors had the histiocytoid pattern, and seven of these had presented diagnostic problems histopathologically, the misinterpretations resulting in diagnoses of xanthelasma, xanthoma, histiocytoma, histiocytosis, granular cell myoblastoma, and possible blastomycosis, all of which reflect the histiocytic appearance of these mucus‐secreting metastatic adenocarcinomas.

Rhabdomyosarcomatous differentiation in malignant intraocular medulloepitheliomas

Four intraocular medulloepitheliomas have been found to contain rhabdomyosarcomatous elements. In three tumors, typical rhabdomyoblasts containing myofibrils with cross striations could be easily identified by light microscopy, but, in one case, electron microscopy was necessary to establish the rhabdomyosarcomatous nature of large ganglioform cells contained among the medulloepitheliomatous elements. In two of the tumors, there were islands of hyaline cartilage within the tumor and in one of the others, in which only a portion of the original specimen was available for study, and in which no cartilage was found in the primary tumor, the neoplasm recurred in the orbit as a chondrosarcoma. In all cases, there was a remarkable blending of all components of the tumors—the medulloepitheliomatous, the mesenchymal, and the undifferentiated. Some suggestions as to the embryogenesis of these teratoid medulloepitheliomas are discussed.

Alveolar soft-part sarcoma of the orbit: A clinicopathologic analysis of seventeen cases and a review of the literature

This is a clinicopathologic study of 17 cases of alveolar soft-part sarcoma of the orbit. The mean age of patients was 23 and the median was 18 (range, 11 months to 69 years); 13 patients (76.5 per cent) were women and four were men (ratio, 3.25:1). The right and left orbits were equally involved (eight patients each), and in one the laterality was not specified. Histologically, the tumors had a distinctive organoid pattern outlined by thin-walled capillaries and were composed of nests of large polyhedral cells with abundant, finely granular, acidophilic cytoplasm. Approximately two thirds of the tumors had diagnostic PAS-positive diastase-resistant crystalline structures. Histologically, the differential diagnosis included nonchromaffin paraganglioma, granular cell tumor, metastatic renal cell carcinoma, vascular tumor, alveolar rhabdomyosarcoma, and amelanotic malignant melanoma. Electron microscopic studies of one tumor disclosed mitochondria with abnormal cristae, increased amounts of glycogen, and cytoplasmic crystalline structures with a periodicity of 8 to 10 nm. Smaller membrane-bound electron-dense granules appeared to be precursors of the crystals. Follow-up studies showed that eight patients were alive and well (median follow-up period, 11.4 years). Six of the eight patients at the time of diagnosis were 20 years of age or younger. A ninth patient was alive and well 13 years after excision of the orbital mass and four years after bilateral thoracotomy with resection of nine pulmonary nodules. Two patients died as a result of metastatic disease, one 14 years and the other 21 years after initial orbital surgery. Two patients died of other causes, one of whom had pulmonary metastases at autopsy. The follow-up period on two recent cases was less than three years, and two patients were lost to follow-up. The disease pursued an indolent clinical course. Surgery offers the best chance to control the disease.

Fetal rhabdomyoma.An analysis of nine cases

Nine cases of a distinctive yet uncommon soft tissue tumor are reported with the pertinent clinical and pathologic features. The tumor, which was usually present shortly after birth, occurred, with one exception, in children 3 years of age and under. Male patients outnumbered females 8:1. The lesion was most common in the subcutaneous tissues of the head and neck region, especially the posterior auricular area. The histologic features revealed myoblasts in different stages of differentiation bearing a striking resemblance to fetal skeletal muscle in the 6th to 10th week of development. Electron microscopic study of formalin‐fixed paraffin‐embedded tissue demonstrated unequivocally the striated muscle origin of the tumor. After local excision, none of the tumors recurred in the follow‐up interval, which ranged from 1 to 10 years (median survival 5.5 years). This tumor can be clearly distinguished from the adult rhabdomyoma. The only other neoplasm that may produce some confusion in the differential diagnosis is the well‐differentiated embryonal rhabdomyosarcoma. A means of separating these two entities is discussed.

Corneal Microsporidiosis: A Case Report Including Ultrastructural Observations

Stromal keratitis and iritis developed in the left eye of a healthy 45-year-old man with no history of ocular disease, trauma, or contact lens wear. The clinical course over a 2-year period was characterized by progressive central disciform keratitis, recurrent anterior stromal patchy infiltration, and iritis which was partially controlled with topical corticosteroids and broad-spectrum antibiotics. Results of bacterial, viral, fungal, and chlamydial cultures were negative. Results of histopathologic examination of a corneal biopsy specimen and, later, a penetrating keratoplasty specimen showed many extracellular and intracellular spores in degenerating keratocytes. By electron microscopy there were encapsulated oval structures measuring approximately 3.5 to 4 microns in length x 1.5 microns in width. Mature spores had well-developed cell walls that contained two abutted nuclei (diplokaryon) and a redundant polar tubule with six coils. These structures are characteristic of a protozoa in the genus Nosema.

Melanotic schwannoma of soft tissues. Electron-microscopic observations and review of literature

This is a report of an unusual case of melanotic schwannoma located in the soft tissue of the shoulder. By light microscopy, the partially pigmented, encapsulated mass showed tumor lobules displaying an organoid pattern and benign cytologic features. By electron microscopy, the tumor cells disclosed numerous interdigitating blunt cytoplasmic processes invested by a thin, continuous basal lamina with formation of mesaxons and moderate amount of glycogen as well as immature and mature melanosomes. The patient was reported to be alive and well 7 years after local excision of the mass. We also reviewed 21 additional cases from the literature. The most frequent sites of involvement were: the spinal nerve roots and adjacent structures (12 cases), soft tissues (five cases), heart (one case), esophageal wall (one case), acoustic nerve (one case), and mandible (one case). Twelve patients were alive and well (follow-up periods ranging from 9 months to 13 years after surgical resection), and one died of surgical complications. Eight patients were lost to follow-up. Three patients had local recurrences due to incomplete excision of the mass. Ten tumors were studied electron microscopically and displayed characteristic features of both Schwann cells and melanocytes. This unique dual differentiation probably reflects their common ancestry from neural crest cells.