Clinically-occult mixed cellularity Hodgkin's disease with Charcot-Leyden crystals.

Charcot-Leyden crystals (CLC) are rarely described in tissue. Because of the derivation of CLC from eosinophils, and the antineoplastic functions that eosinophils effect, it is plausible that CLC in neoplastic tissue specimens may be significant. We recently encountered a case in which Hodgkin's disease and CLC were unexpectedly found. We reviewed 31 cases of Hodgkin's disease for CLC and sought relationships between CLC incidence and morbidity or mortality. While various grades of eosinophilia were represented, CLC were encountered only in the case reported. The role of eosinophils and CLC in Hodgkin's disease is enigmatic. With clinicopathologic correlations from additional patients, it may be determined that CLC play a role in the natural history or prognosis of Hodgkin's disease.

Paget's disease in an adolescent arising in a supernumerary nipple.

Paget's disease of the breast generally affects middle-aged and older females. In the majority of cases, it is considered to be the result of epidermal spread from a contiguous mammary duct carcinoma. We report the first case in an adolescent female with an accessory nipple. The histomorphology and immunophenotypic findings support the hypothesis of in situ transformation and suggest a histologic spectrum exists in terms of cytologic atypia.

Alopecia neoplastica simulating alopecia areata and antedating the detection of primary breast carcinoma.

A woman with no previous history of breast carcinoma presented with focal hair loss, which was presumptively diagnosed as alopecia areata. After treatment failures, a scalp biopsy was performed, which subsequently led to the diagnosis of breast carcinoma. This case illustrates the subtle course which breast cancer can take and the insidious manner in which it may present. It alerts clinicians and pathologists to the possibility of secondary causes, including malignancy, in the differential diagnosis of alopecia refractory to usual treatments.

Pulmonary phaeohyphomycosis due to Xylohypha bantiana.

We encountered a rare case of pulmonary phaeohyphomycosis due to Xylohypha bantiana documented by culture. This dematiaceous (darkly pigmented) fungus is primarily neurotropic. It usually produces phaeohyphomycosis of the central nervous system but may also involve the skin and subcutaneous tissues. The patient, a 49-year-old woman with a history of steroid-treated inflammatory bowel disease, was found to have a lung nodule consisting of granulomas that contained dark hyphal fragments that stained positively with the argentaffin reaction. Surgical excision was curative and appears to be the treatment of choice.

Stage I and II non-Hodgkin's lymphomas: long-term results of radiation therapy.

One hundred and sixteen patients with Stages I and II non-Hodgkin's lymphomas were treated with curative radiotherapy between 1964 and 1977. The initial biopsy material was classified according to the criteria of Rappaport et al. All the patients except six were followed for a minimum of 8 years or until death with ninety-two patients having been followed for 10 or more years. Histological pattern was found to be an important prognostic factor with better survival in patients with nodular histology. There were forty-three patients with an initial involvement of an extra-nodal site forming 37% of the total group. Stage I extra-nodal lymphomas had a survival and recurrence-free survival rates of 80% and 74% respectively, similar to those of nodal lymphomas. Stage II patients in both groups did worse than those with Stage I. Survival rates and disease-free survival rates for different histologic types and their pattern of recurrence are presented.

Solitary tumors of meibomian gland origin and Torre's syndrome.

We examined ten patients with solitary sebaceous gland tumors of the eyelid for Torre's syndrome (sebaceous gland tumors and visceral malignancy). In all of the benign and hyperplastic sebaceous lesions, an associated visceral malignancy was noted (predominantly colonic). Only one patient with a sebaceous carcinoma had an associated visceral malignancy. Our data suggest that the presence of a solitary sebaceous gland lesion of the eyelid excluding sebaceous carcinoma is clinically significant and should warrant a thorough history and physical examination to exclude a visceral malignancy, primarily of the gastrointestinal tract. The solitary sebaceous lesions excluding sebaceous carcinoma may represent part of the histologic spectrum associated with Torre's syndrome.

Cutaneous cholesterol emboli with infarction clinically mimicking heparin necrosis--a case report.

Cutaneous necrosis secondary to anticoagulation (heparin and warfarin) and cholesterol (atheromatous) emboli can be similar clinically and histologically. A unique case is reported of cholesterol emboli clinically mimicking heparin necrosis. The patient was a 57 year old white male who underwent coronary angioplasty and was treated with intravenous heparin. Shortly after he developed large ecchymotic areas at the level of the umbilicus and distally which progressed to eschar. Renal failure ensued and he died secondary to stress ulcers. Cholesterol emboli were demonstrated in small muscular arteries of the dermis and panniculus along with a full thickness infarct. Because of the increasing popularity of angioplasty utilizing a retrograde femoral approach plus heparin anticoagulation, it is important to recognize the difficulty in making a clinical diagnosis when cutaneous necrosis supervenes. An incisional biopsy of the skin is recommended.

Composite lymphoma with immunoblastic features and Langerhans' cell granulomatosis (histiocytosis X).

A 50-year-old male with a history of tonsillar and right axillary lymph node enlargement due to atypical lymphoid hyperplasia presented two years later with marked bilateral axillary and inguinal lymphadenopathy. The lymph node biopsy showed a composite lymphoma (follicular, mixed, small and large cell plus B-immunoblastic sarcoma) with associated focal Langerhans' cell granulomatosis (LCG) (Histiocytosis X). The diagnosis of composite lymphoma was supported by the immunohistochemical demonstration of two different monoclonal patterns in the follicular and diffuse areas. The typical Birbeck's granules were demonstrated ultrastructurally in LCG areas, which also stained with S-100 protein. LCG may coexist with malignant lymphoma, however, it appears to be confined to the neoplastic nodes with no tendency to systemic spread. It is important to recognize this association so that the impact of this apparently benign lesion (LCG) not be overestimated and that the subsequent management of the patient be directed according to the type of the coexisting malignant lymphoma.

Clear cell sarcoma and selective IgM deficiency: a case report.

A case of clear cell sarcoma of tendons and aponeuroses and a co-existent IgM deficiency is reported. The tumor arose in the Achilles tendon with metastases to the skin, bone, and lymph nodes. The tumor, examined by light and electron microscopy, consisted of glycogen-containing clear cells with melanotic and amelanotic features. There was no detectable serum IgM. The IgA levels were normal and IgG levels were elevated. Peripheral blood lymphocytes contained a normal amount (4.5%) of IgM-bearing cells. Cultured mononuclear cells from the patient suppressed production of IgM by normal lymphocytes, suggesting a role of suppressor cells in the IgM deficiency. The co-existence of soft tissue sarcomas and immunoglobulin deficiency states is reviewed.

Recurrence of Wilms tumor--twenty-three years later.

A case of Wilms tumor recurrent twenty-three years after the initial nephrectomy and subsequent radiotherapy is presented. Late recurrence of Wilms tumor is rare, and one must postulate a breakdown in the host's immune surveillance system to explain such an unusual event.

The otolaryngologic manifestations of malignant lymphoma with a high content of epithelioid histiocytes (Lennert's lymphoma).

Malignant lymphoma with a high content of epithelioid histiocytes is a rare non-Hodgkin lymphoma first described by Lennert and Mestdagh (Lennert's lymphoma) in 1968. Distinguishing characteristics include frequent involvement of the nasopharynx, Waldeyer's ring, and cervical lymph nodes. Of the four patients included in this report, three had Waldeyer's ring involvement and four had cervical lymphadenopathy. Our experience and that of others demonstrate the unpredictable and variable course of the disease, as well as the inconsistent response to various therapeutic modalities; especially interesting is the remission obtained in one patient treated with corticosteroids alone, in contrast to the others who followed a rapid downhill course to death in spite of (or as a complication of) chemotherapy and radiation. The role of the otolaryngologist in the diagnosis and management of the disease is emphasized.

Prolapse of the laryngeal ventricle.

Many varied lesions may protrude into the laryngeal lumen from between the true and false vocal cords. These protrusions can be precisely diagnosed only by biopsy. Prolapse of the laryngeal ventricle is one of these lesions; it is a distinct clinicopathologic entity, a primary lesion unrelated to other laryngeal or systemic disease. Eversion of the ventricle (or saccule) is a similar protrusion that is secondary to pulsion or traction by an associated laryngeal lesion. Other lesions that may produce similar clinical findings and gross appearance include benign tumors, cysts, and, rarely, squamous cell carcinoma. Biopsy is therefore mandatory to plan rational treatment. A review of 26 patients who had been diagnosed as having prolapse resulted in the reclassification of 20 cases of prolapse and six cases of eversion. Prolapse is possibly unrelated etiologically to cough, or chronic laryngeal or respiratory tract infection. The histopathologic data suggest further that prolapse is a result of fatty infiltration, edema, and inflammation of the periventricular tissue above the ventricle, which cause the tissue to enlarge and protrude into the laryngeal lumen from between the true and false vocal cords.

Lymphoid hyperplasia of larynx simulating neoplasia.

A rare case of lymphoid hyperplasia of the adult larynx (hyperplasia of the laryngeal tonsil) is reported. While this benign lesion recurred following surgical excision, the patient has been followed for more than two years without additional therapy and is asymptomatic. Seven additional cases of laryngeal lymphoid hyperplasia in the English literature are reviewed. The problems related to inadequate sampling (biopsy) and the histologic criteria for diagnosis are emphasized. To define the pathogenesis and ultimate biologic potential of this lesion, more cases must accrue and be studied with a long follow-up.

Early nodal and extra-nodal non-Hodgkin's lympnomas.

There were 54 Stage I and 38 Stage II patients with non-Hodgkin's lymphomas treated with curative radiotherapy between 1964 and 1975. The initial biopsy material was reclassified according to the criteria of Rappaport el at.10 The frequency of nodular vs diffuse histological pattern was 26% vs 74%. The survival and recurrence free survival were significantly superior for the nodular pattern as compared with diffuse in both Stages I and II. The sites of first recurrence were evaluated. Fourteen out of 20 Stage I and 18 out of 26 Stage II patients recurred in one of the nodal areas only as their first site of recurrence. In 50%, this was in contiguous lymph node areas only. Extra-nodal non-Hodgkin's lymphomas survival and recurrence-free survival rates at five years were identical.