Multifocal extraosseous eosinophilic granuloma of the head and neck.

Multifocal eosinophilic granuloma (Hand-Schüller-Christian disease) confined to soft tissues in the head and neck region is described in a man, 32 years of age. The lesions were detected at varying time intervals within a period of 14 months. They were located in the gingivae, the submaxillary salivary glands, the thyroid gland, and in the dermal lining of the external auditory meatus. Clinical, histologic, ultrastructural, and immunohistochemical findings are presented and discussed. Some of these findings go along with the tenet that the typical proliferating histiocyte in eosinophilic granuloma is a pathologic Langerhans' cell, or a close kindred to it.

Non-Hodgkin's lymphoma complicating acute lymphoblastic leukemia in remission.

Second malignancies occurring in patients cured of childhood acute lymphoblastic leukemia (ALL) are rare but increasingly recognized. There are only three reported cases of non-Hodgkin's lymphoma complicating ALL in remission. We report here another case of B-cell-type large-cell lymphoma, presenting in the uterus of a 24-year-old woman, 16.5 years after the diagnosis of ALL was made. Although immune markers of the ALL were not available, it was thought to be unlikely that the second malignancy was a relapse of the original tumor. Due to the long latency period and the minimal exposure to alkylating agents, the role of chemotherapy seems to be insignificant in this case. The development of a second malignancy could be a manifestation of the basic susceptibility to cancer.

Adenocarcinoma of the epiglottis. Report of a case and review of the literature.

The larynx is rarely the site of an adenocarcinoma. About 60 patients were described in the literature, and the epiglottis was primarily involved in only three. We studied a fourth case in a 77-year-old man. Adenocarcinoma of the larynx is a highly lethal neoplasm that tends to spread to both regional lymph nodes and distant sites. Despite radical surgical measures, the cure rate is low.

A benign course of multicentric Castleman's disease with involvement of the spleen and bone marrow.

The multicentric variant of Castleman's disease (MCCD) is associated with a rapidly progressive and fatal course. The case described herein manifested unique clinical and histological features. Initial presentation as isolated splenomegaly was subsequently followed by widespread organ involvement, including lymph nodes and bone marrow. In spite of this, the patient had a very benign course of her disease. The case serves to expand even further the already wide clinical spectrum of Castleman's disease.

Nephrogenic adenoma: benign proliferative lesion of urothelium.

Three new cases of a rare benign proliferative lesion of the urothelium (nephrogenic adenoma) are hereby added to the existent 58 reported cases. All 3 lesions occurred in the bladder. Two of the 3 cases presented with a history of surgical interventions on the bladder, and the third patient had a history of chronic cystitis and pyelonephritis, and was on chronic dialysis. Diagnosis was made on histologic examination of cystoscopic biopsy of the lesions. Transurethral resection and/or fulguration was the treatment of choice. There was no recurrence of the lesions on follow-up cystoscopic examination.

Angioimmunoblastic lymphadenopathy with scleromyxedema-like lesions and serum monoclonal protein.

A patient was followed up for 5 yr for an illness diagnosed histologically as angioimmunoblastic lymphadenopathy. His serum contained a monoclonal protein of the IgM type, and biopsies from papular skin lesions revealed scleromyxedema-like features. The unusual association of this kind of cutaneous lesion with angioimmunoblastic lymphadenopathy and a monoclonal IgM is discussed.

Neuropathic form of arthrogryposis multiplex congenita. Report of 3 cases with complete necropsy, including the first reported case of agenesis of muscle spindles.

In 3 infants with arthrogryposis multiplex congenital (AMC) complete necropsy, including removal of the entire spinal cord, was performed. Histologically, spinal type (neurogenic) atrophy of skeletal muscles in conjunction with spinal motor neurone depletion, unaccompanied by noticeable gliosis, were the most preminent features common to all cases. In addition to these, one infant (Case 1) showed total absence of muscle spindles as evident from the examination of several hundred step-serial paraffin sections covering 11 different levels in 36 samples taken from a wide range of skeletal muscles of both upper and lower extremities. This is believed to be the first published case of agenesis of muscle spindles.

Synovial sarcoma of the hypopharynx.

Primary synovial sarcoma of the hypopharynx is an extremely rare neoplasm. Only 16 cases could be traced in the literature so far. An additional case, in a 29-year-old male patient, is presented. Progressive disturbance of deglutition and speech, chocking sensation, and mild respiratory distress were his original complaints. Our case differs from those previously reported in that a) the tumor was pedunculated, and the site of its attachment could be very well-defined, and b) unusual intratumoral destructive hemorrhage turned the case into an emergency. The pedicle was radically excised together with part of the grossly uninvolved underlying tissue, and the tumor was removed in toto. The histological findings are described. The patient is under observation. No recurrence has been noticed 11 months after surgery, and there are no signs of metastasis. The origin of the tumor and its treatment are discussed.

Solitary lung involvement from clear cell carcinoma of the endometrium.

Clear cell (Müllerian) carcinoma of the endometrium is relatively seldom reported. It is a disease with an unfavorable prognosis. A patient in whom a lung metastasis was diagnosed accidentally and excised is herein presented. The patient is alive five years after hysterectomy. The histopathologic pattern of the tumor is briefly described, and some of the clinical aspects of endometrial carcinoma are discussed.

Primary malignant melanoma of the small intestine and the APUD cell concept.

A primary melanoma of the small intestine is reported. A possible explanation of its origin according to the APUD cell concept is suggested. This concept may lessen the doubts and confusion whenever an intestinal melanoma is discovered and a primary tumor at one of the more common sites can reasonably be excluded.

Unusual amyloid bodies in human liver. Ultrastructural and freeze-etching studies.

An unusual presentation of amyloidosis in the liver is described in two patients with chronic pulmonary disease. Amyloid bodies, ranging from 3 to 20 microns in diameter and staining positive for Congo red, were found to be deposited between or within hepatocytes and, therefore, to be in a more direct spatial relationship to epithelial than to Kupffer or sinusoidal lining cells. The freeze-etching technique revealed that the average diameter of the amyloid fibrils was 15 nm and not 7 to 10 nm. This may be due to the fact that this technique avoids shrinkage artefacts which follow fixation and dehydration.

Gold tracer studies of muscle regeneration.

Rabbits were injected with soluble gold, and cold injury was inflicted upon their extraocular muscles. Following the typical freeze-thaw damage in which the myofiber basement membrane is preserved, a polymorphonuclear granulocyte exudation occurred. At this stage only a few interstitial cells with long processes contained gold. At one and two days abundant mononuclear cells with gold were observed at the site of injury. In subsequent stages of muscle regeneration the amount of tracer decreased but myoblasts, and even fully formed myotubes, often contained detectable gold. Some data on "in vivo" gold salt kinetics and the specificity of gold localization were utilised in the study of the relation between mononuclear interstitial cells and myoblasts.