Sudden and unexpected infant death due to occult lymphoblastic leukaemia.

A 16-week-old boy was found moribund in his crib. At autopsy widespread infiltration of multiple organs by lymphoblastic leukaemia (pre-B cell type) was found. Death was attributed to extensive myocardial infiltration by leukaemic cells. There was no associated intracranial haemorrhage. Home video footage taken on the day before death revealed a non-distressed and relatively normal-appearing infant. This case demonstrates an extremely rare cause of unexpected infant death and emphasizes the need for full autopsy examination in such cases. It also confirms that certain infants may have minimal or no symptoms of illness, even in the presence of severe, systemic, life-threatening disease.

Granulomatous lobular mastitis.

Two cases of benign lobular granulomatous mastitis, both clinically felt to be carcinoma, are reported. One patient was cured by local excision of the mass while the other was cured by oral prednisolone, demonstrating the variable ways to treat this entity. The clinical and histological features were similar to those noted in previous reports. Immunological studies revealed predominantly stromal T lymphocytes and only duct intra-epithelial T lymphocytes.

Tissue response to artificial hormone replacement implant.

The histological changes relating to 2 separate artificial hormone replacement implants in a 34 yr old female are presented. Both sites essentially showed a predominant lymphohistiocytic reaction with fibrosis. One implant was intact while the other had disintegrated.

Postimmunization (vaccination) injection-site reactions. A report of four cases and review of the literature.

Four patients each had a single subcutaneous nodule at the site of a previous vaccine injection; three after injection of diphtheria, tetanus, and pertussis vaccination and one after tetanus toxoid vaccination. Presentation was with a mass 4-22 months after vaccination at the site of injection. Histologically, three patients had a necrotizing granulomatous reaction with a surrounding infiltrate of lymphocytes, plasma cells, histiocytes, and associated fibrosis. The fourth patient demonstrated a lymphohistiocytic reaction with a predominance of histiocytic cells as well as associated plasma cells, fibroblasts, and fibrosis. The lymphoid infiltration in these reactions showed a predominance of T-lymphocytes over B-lymphocytes. Aluminum was demonstrated in necrotic foci, inflammatory stroma, and the granular cytoplasm of histiocytes with the aid of solochrome azurine and solochrome cyanine stains as well as by energy-dispersive x-ray microanalysis. The reactions are thought to be immunologic (hypersensitivity) reactions associated with the aluminum contents of the preparation.

Intra-abdominal desmoplastic small round cell tumour.

Intra-abdominal desmoplastic small round cell tumour is a rare tumour which has only recently been described. It demonstrates epithelial, mesenchymal and neural features and has an aggressive clinical course. We report a case of a 15 year old male with this tumour who, after debulking surgery and chemotherapy, relapsed with disease 1 month later. After further debulking surgery, extensive chemotherapy and bone marrow transplantation he relapsed with disease 5 months later and died. Total survival was 20 months.

Small cell (neuroendocrine) carcinoma of the vagina.

We report a case of vaginal small cell (neuroendocrine) carcinoma. Immunostaining for neuron-specific enolase, PGP 9.5, chromogranin, synaptophysin, Leu 7 and cytokeratin was positive. Neurosecretory granules were found by electronmicroscopy. There was local recurrence and regional lymph node metastases. The patient survived for 10 months following local surgical therapy only.

Extradural malignant lymphoma associated with spinal cord compression.

Cord compression was noted in 26 patients with extradural malignant lymphoma. There were 19 cases of nonHodgkin's lymphoma and seven cases of Hodgkin's disease. Eighteen cases of non-Hodgkin's lymphoma had intermediate or high grade malignancy types according to the Working Formulation Lymphoma Classification. These patients represented 28% of all extradural malignant tumours seen at the Royal Adelaide Hospital (RAH) and 2.5% of all patients with malignant lymphoma, during an 11 year period. The patients were classified in three presenting groups: Group A, six patients with primary extranodal extradural lymphoma; Group B, nine patients with both extradural lymphoma and disseminated disease at initial presentation; and Group C, 11 patients who developed extradural lymphoma during the course of established disease. Only classification by groups appeared to affect survival time. Group A had the most favourable prognosis, with a 5 year survival of 83%. Five of the Group A patients had a relapse of lymphoma; four at distant sites and one in the retroperitoneum. Laminectomy was essential to provide a diagnosis in Group A patients. The most useful warning symptom of impending spinal cord compression was back and/or radicular pain, which preceded neurological deficit either by days or by up to 5 years. Plain spinal X-rays were abnormal in 64% of cases, emphasizing the value of this simple procedure.

Localized herpes simplex lymphadenitis: report of three cases and review of the literature.

This report describes three cases of localized or regional herpes simplex virus lymphadenitis. Of the two patients who presented with inguinal lymphadenopathy, one had a localized rash prior to the development of lymphadenopathy, and the other developed a genital rash 2 days after lymph node biopsy. The third had occipital lymphadenopathy and concurrent localized occipital herpetic folliculitis. Localized herpes simplex virus lymphadenitis appears to be a specific clinicopathological entity. Histologically, it is characterized by varying degrees of follicular and paracortical hyperplasia, a prominent immunoblastic response and perinodal inflammation. Viral inclusions with or without focal necrosis in the lymph node may be seen, and immunostaining for viral antigen may be seen in paracortical cells.

Adenoid cystic carcinoma in a juvenile male breast.

Adenoid cystic carcinoma of the breast is uncommon and comprises less than 1% of all mammary cancers. Its occurrence in the male breast is very rare. We describe the first case of adenoid cystic carcinoma in a juvenile male breast. The patient is alive and well 2 1/2 yrs following subcutaneous mastectomy. The details of the 4 previously reported cases of the same tumor in male breasts are reviewed.

Carcinoid tumor occurring in a mature testicular teratoma.

A rare case of a primary testicular carcinoid tumor, occurring in a mature teratoma from a 33 year old man, was examined histochemically, immunohistochemically and by electron microscopy. Both argyrophil and argentaffin reactions were positive. Immunoreactive cells for 5-hydroxytryptamine (5-HT) and gastrin were present. Electron microscopy demonstrated neurosecretory-type granules. The English literature was reviewed and this revealed at least 6 similar cases.

Right-sided diverticular disease of the colon: a morphological analysis of 16 cases.

Right-sided diverticular disease is uncommon and represents a heterogeneous group of cases with varying clinical presentation often confused with other disorders. Based on number, size, distribution and histological appearance of the diverticula, four distinct groups were identified, each with a distinct clinical presentation. Six patients had solitary false diverticula mimicking acute appendicitis and at operation were found to have inflamed caecal masses. Five patients had diverticula formed on the basis of defects in the muscularis propria. The mean age of these patients was 30 years older than the previous group and all of them presented with rectal haemorrhage, often confused with angiodysplasia clinically. At laparotomy the external appearance of the bowel was unremarkable. Three patients had diverticular disease similar to that seen in the left side of colon. Two patients had true congenital caecal diverticula confirming that the minority of caecal diverticula conforms to this group.

Therapy for childhood soft-tissue sarcomas other than rhabdomyosarcoma: a review of 62 cases treated at a single institution.

The rarity and diverse characteristics of the nonrhabdomyosarcomatous soft-tissue sarcomas (NRSTS) in children have hindered study of their clinical presentations and response to therapy. Here we describe the findings of a retrospective analysis of 62 cases of NRSTS seen in a single institution from 1962 through 1983. The most common histopathologic diagnosis was synovial sarcoma, occurring in 18 patients, followed by malignant schwannoma in 12. The median age at diagnosis was 11 years (range, 2 months to 20 years). Anatomic sites of primary tumors were the trunk (28), extremity (24), and head and neck (10). Of the 31 patients whose tumors were completely resected, 26 (84%) survive with no evidence of disease. Postoperative chemotherapy, administered to nearly one half of this group, did not produce any demonstrable gains in survival. Only one of the 26 patients with local or metastatic gross tumor after resection survives. We conclude that an aggressive surgical approach is imperative in patients with NRSTS and that the contribution of other treatment modalities needs to be defined in a collaborative group trial.