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Biomedical subjects

L E Zimmerman

Publications and source records attributed to L E Zimmerman.

At least 37 records · Page 2Linked to original sources
PubMed1988

Cutaneous malignant melanoma in survivors of heritable retinoblastoma.

Six survivors of bilateral retinoblastoma developed cutaneous malignant melanoma 20 to 51 years (average, 31 years) after initial therapy for the ocular tumor. Five patients received radiation therapy to the orbital area. In two patients the cutaneous malignant melanoma developed in the field of irradiation. Two patients developed multiple cutaneous melanomas and are thought to have the dysplastic nevus syndrome. At this writing two patients are alive and well after wide resection of their skin tumors. A review of the literature suggests that cutaneous malignant melanoma accounts for about 7% of second malignant neoplasms in survivors of heritable retinoblastoma.

Adult↗
PubMed1988

Sinus histiocytosis presenting as an epibulbar mass. A clinicopathologic case report.

Sinus histiocytosis is a well-recognized condition that mainly affects children in the first decade of life. Although this usually benign disease often features massive cervical lymphadenopathy, numerous extranodal sites of involvement can be seen. We describe a new presenting manifestation for sinus histiocytosis. A 13-month-old infant developed a rapidly growing unilateral epibulbar tumor that involved the cornea. A complete physical examination revealed only mild inguinal lymphadenopathy. A diagnosis of sinus histiocytosis was made by histologic examination of the lesion after surgical excision.

Corneal Diseases↗
PubMed1988

Incidence of second neoplasms in patients with bilateral retinoblastoma.

The cumulative incidence of second neoplasms in 215 patients with bilateral retinoblastoma was calculated using the life-table method. Second tumors developed in 4.4% of the patients during the first 10 years of follow-up, in 18.3% after 20 years, and in 26.1% after 30 years. The 30-year cumulative incidence was 35.1% for the 137 patients who received radiation therapy compared with an incidence rate of 5.8% for the 78 patients who did not receive radiation. In the 137 patients who received radiation, second tumors developed both inside and outside the field of therapy. There was a 30-year incidence rate of second tumors of 29.3% within the field of irradiation and 8.1% outside the field. The rate outside the field of irradiation (8.1%) was similar to that observed in nonirradiated patients (5.8%). Our findings indicate that carriers of the retinoblastoma gene have an increased incidence of second tumors, and that the incidence rate is further increased in patients who receive radiation therapy.

Child, Preschool↗
PubMed1988

Unusual eyelid tumors with sebaceous differentiation in the Muir-Torre syndrome. Rapid clinical regrowth and frank squamous transformation after biopsy.

An unusual eyelid tumor with sebaceous differentiation developed in two 70-year-old men and each had an earlier history of one or more colonic carcinomas--the so-called Muir-Torre syndrome. These eyelid tumors were both behaviorally and histopathologically different from previously documented cutaneous neoplasms in this syndrome. In the first case, a sebaceous adenoma took multifocal origin from the epidermis and exhibited an interanastomosing retiform growth pattern in the underlying dermis; the lesion rapidly recurred as a keratoacanthoma after subtotal excision. In the second case, a conjunctival tumor with features of a poorly differentiated squamous cell carcinoma manifested focal evidence of sebaceous differentiation. Again, after an incisional biopsy, the latter tumor rapidly regrew and transformed into a well-differentiated squamous cell carcinoma with massive central necrosis. Although sebaceous carcinoma of the eyelids does not appear to presage the Muir-Torre syndrome, any benign sebaceous or transitional squamo-sebaceous neoplasm should be considered a possible manifestation of this syndrome. Both clear-cut benign and transitional sebaceous neoplasms should also be recognized as having the potential to undergo an ominous clinical regrowth upon subtotal excision and a complete squamous transformation.

Adenoma↗
PubMed1988

Early onset acute orbital involvement in childhood acute lymphoblastic leukemia.

A 5-year-old girl with acute lymphoblastic leukemia undergoing induction chemotherapy experienced acute proptosis while agranulocytotic and febrile. Orbital biopsy showed leukemic infiltration, and complete resolution was achieved with local irradiation and chemotherapy. Early onset orbital involvement is highly unusual in acute lymphoblastic leukemia. In the setting of agranulocytosis and fever, rapidly enlarging intraorbital masses require urgent ophthalmologic attention. Immediate biopsy is indicated to distinguish between several treatable conditions including opportunistic infection, hemorrhage, and neoplastic infiltration.

Acute Disease↗
PubMed1988

Atypical cases of sinus histiocytosis (Rosai-Dorfman disease) with ophthalmological manifestations.

Sinus histiocytosis with massive lymphadenopathy is a non-neoplastic disease of unknown cause observed mainly in children with markedly enlarged cervical or other lymph nodes. We have reported two very atypical cases with extranodal manifestations that required ophthalmological consultation. Neither patient had significant lymphadenopathy. One patient, who had tumors of all four eyelids as well as lesions of the nose and larynx, was 68 years old when first examined, and 70 at the time of his last surgical procedure. The other patient, a 13-month-old child, had a unique corneoscleral lesion as his only clinical manifestation. The excised tissues obtained from the eyelids and larynx in case 1 and from the corneoscleral lesion in case 2 revealed characteristic histopathologic features of sinus histiocytosis (Rosai-Dorfman disease), including strongly positive immunoperoxidase staining for S-100 protein in the cytoplasm of most of the histiocytes.

Age Factors↗
PubMed1988

Histopathological changes in the eyes of mangabey monkeys with lepromatous leprosy.

Leprosy is the third leading cause of preventable blindness; however, little is known about the spread of infection to the eye. We have studied the eyes of three sooty managabey monkeys. Two were experimentally infected with Mycobacterium leprae; the third was not infected. In one of the infected animals there was histopathological evidence of lepromatous leprosy as evidenced by a chronic inflammatory infiltrate at the limbus, and detection of acid-fast bacilli in the corneal stroma, blood vessel walls, and corneal nerves. The latter were damaged as a result of the bacillary invasion. Electron microscopy revealed involvement and distortion of keratocytes with M. leprae and invasion of the corneal stroma by macrophages containing bacilli. Both infected animals showed focal collections of lymphocytes in the superficial stroma of the conjunctiva and in the ciliary body. This is the first report of the ocular manifestations of leprosy in any primate, including man, in which the duration of infection is known.

Animals↗
PubMed1987

Immunopathology and electron microscopy of Acanthamoeba keratitis.

In order to evaluate the local cellular immune response to Acanthamoeba infection we performed immunohistochemical examinations of the corneal buttons of two patients with Acanthamoeba keratitis. We found that the corneal stroma was infiltrated with polymorphonuclear leukocytes and HLA-DR positive macrophages that appeared to be stromal keratocytes by light microscopy. Despite the presence of chronic inflammation in both patients, no stromal lymphocytes were seen in one patient and a sparse lymphocytic infiltrate was seen in the other patient. Electron microscopy confirmed the presence of macrophages, neutrophils, and Acanthamoeba organisms in these two patients.

Adult↗
PubMed1987

Metastatic colloid carcinoma versus primary carcinoma of the ciliary epithelium.

Ocular metastases developed from breast carcinomas in two women 7 and 19 years after their mastectomies. They were both ciliary body metastases that had eroded through the root of the iris to present as unifocal globular, gelatinous (colloid) masses in the anterior chamber, and were amenable to local surgery. Histopathologically, small cellular clusters were widely separated in a sea of mucin; the tumor cells failed to display marked pleomorphism or mitotic activity. In each case, the distinction from a primary mucinous ciliary epithelial neoplasm had to be made both clinically and pathologically. For comparison, the authors also report a unique primary ciliary carcinoma that caused intractable glaucoma by spreading diffusely throughout the iris, ciliary body, and anterior chamber angle, and that necessitated enucleation. The tumor cells failed to produce hyaluronic acid but elaborated a mucosubstance which was histochemically indistinguishable from that of the metastatic carcinomas. This primary neoplasm, however, exhibited the following histologic differences from the metastases: more architectural variability, including garlands and festoons of cells not forming lumens that were suspended in a mucinous matrix; much more abundant intracellular mucin; foci of sheet-like and pleomorphic cellular proliferations with mitotic activity; and partial replacement of the ciliary processes by a comparatively benign-appearing mucinous columnar epithelium.

Adenocarcinoma, Mucinous↗
PubMed1986

Posterior uveal melanomas in aphakic and pseudophakic eyes.

Of 650 eyes with posterior uveal melanomas that were accessioned at the Armed Forces Institute of Pathology from 1975 to 1983, 28 were aphakic and eight were pseudophakic. In ten of these 36 cases, the cataract had been unilateral. Only two patients had been examined with preoperative A-scan ultrasonography to obtain the axial length of the eye. None of the patients had been examined for possible intraocular tumors by either A- or B-scan ultrasonography. We believe that in many of these cases the tumor was large enough to have been detected at the time of cataract surgery. Thus, if the lens is too opaque for the fundus to be viewed, B-scan ultrasonography should be used before cataract extraction.

Aged↗
PubMed1985

Primary acquired melanosis of the conjunctiva.

Forty-one cases of conjunctival primary acquired melanosis (PAM) were studied to determine the frequency of progression to malignant melanoma and to establish prognostic parameters for progression to melanoma. Two subdivisions were identified: lesions with cytologically atypical melanocytes (PAM with atypia, 28 lesions, 68.3 per cent) and those lacking cytologically atypical melanocytes (PAM without atypia, 13 lesions, 31.7 per cent). None of the lesions of PAM without atypia progressed to melanoma. Thirteen of the 28 lesions of PAM with atypia (46.4 per cent) progressed to melanoma. Progression to melanoma was more frequent in the lesions of PAM with atypia if basilar hyperplasia was not the dominant histologic pattern (90 per cent progression, P = 0.02) or if any epithelioid cells were present (75 per cent progression, P = 0.02). It was not possible to determine which lesions were atypical on the basis of clinical appearance. Lesions at risk for the development of melanoma should be totally extirpated.

Biopsy↗
PubMed1985

Malignant melanoma of the conjunctiva.

One hundred thirty-one cases of conjunctival melanoma in which biopsies had been performed were studied to determine potential factors that might affect outcome in patients with these lesions. Two groups of lesions were identified: those associated with primary acquired melanosis (melanoma with PAM, 98 cases, 74.8 per cent) and those without primary acquired melanosis (melanoma without PAM, 33 cases, 25.2 per cent). The overall mortality rate in the 131 cases was 26 per cent (34 of 131); the mortality rate due to melanoma with PAM was 25.5 per cent (25 of 98), and that due to melanoma without PAM was 27.3 per cent (9 of 33). If PAM was associated with the lesion, the presence of atypical melanocytes within the epithelium (pagetoid invasion) was a sensitive indicator of subsequent metastasis. Tumor thickness may also be useful for predicting subsequent metastases. None of the histologic parameters studied proved useful for predicting outcome in patients who had melanomas without PAM. The presence or absence of nevi had no effect on prognosis.

Adolescent↗
PubMed1985

Cat scratch disease bacilli in the conjunctiva of patients with Parinaud's oculoglandular syndrome.

Unilateral conjunctivitis with regional lymphadenitis has been designated Parinaud's oculoglandular syndrome (POGS). The cause has been attributed to various infectious agents, especially leptothrix and the unidentified agent of cat scratch disease (CSD). In 1983-84 the cause of CSD in lymph node and skin was established as small pleomorphic gram-negative bacilli. We now report identical bacteria in the conjunctival lesions of 9 of 24 patients with POGS. This confirms the belief that the CSD bacillus is a major cause of POGS.

Adolescent↗
PubMed1985

Psammomatoid (juvenile) ossifying fibroma of the orbit.

The clinical and pathologic features in 21 cases of a distinctive solitary fibro-osseous lesion affecting the orbit distinguished histologically by the dominance of numerous small round "psammoma-like" ossicles embedded in a cellular benign spindle cell stroma were studied. The patients were predominantly young, and 19 of the lesions were centered on the orbital plate of the frontal bone or ethmoid. A clinical course of slowly progressive symptoms over several years was typical but not uniform. The most common clinical diagnosis was fibrous dysplasia and mucocele. At least nine patients underwent two or more surgical operations; one patient had six recurrences over a 15-year period. There were no known deaths from the tumor, but its potentially massive size can result in considerable morbidity and cosmetic deformity. Although previously referred to as active juvenile ossifying fibroma, the term psammomatoid ossifying fibroma for this lesion is more descriptive and has historical precedence.

Adolescent↗
PubMed1985

Malignant peripheral nerve sheath tumors of the orbit: a clinicopathologic study of eight cases.

Eight adult patients with malignant peripheral nerve sheath tumors of the orbit are described. Only two of the patients were known to have von Recklinghausen's neurofibromatosis. The typical clinical history included the development of a mass in the superonasal quadrant of the orbit, which was palpable immediately beneath the skin of the lid. There was a definite tendency for the lesions to arise in, or grow along, the supraorbital nerve--including posteriorly through the superior orbital fissure to the Gasserian ganglion, and even as far posteriorly along the trigeminal rootlets to the pons. Delays in pathologic diagnosis, which beclouded the true nature of the process, led to multiple recurrences, eventuating in five known fatalities out of the eight patients. In addition to intracranial extension, pulmonary metastases and regional cervical metastases were encountered. Once recognized for their diagnostic value, the histopathologic patterns are highly distinctive: biphasic populations of spindled and epithelioid cells; sheets of epithelioid cells or clusters demarcated by delicate reticulin fibers or thicker collagenous trabeculae; malignant plexiform patterns; and neurotubular patterns. Pure spindle cell populations were encountered only in the two patients with von Recklinghausen's disease, and in each, either a pre-existent benign neurofibroma or a coexistent plexiform neurofibroma was found in the pathology specimens. The best management of this condition depends upon early clinical and pathological recognition, leading to radical surgery, which usually consists of orbital exenteration combined with intracranial extirpation of as much of the trigeminal nerve as possible. Postoperative radiotherapy and chemotherapy after radical surgery might also be advisable.

Adult↗