Tuberculous bacillemia, hyperpyrexia, and rapid death.

Hyperpyrexia, followed rapidly by multiple organ failure and death, developed in a previously healthy man. Postmortem examination indicated disseminated tuberculosis with adrenal involvement, but also evidence compatible with heat stroke. Mycobacterium tuberculosis was isolated from a routine blood culture. The patient's symptoms may have been the result of his bacillemia or the result of unapparent tuberculous chronic adrenocortical insufficiency that made him unusually sensitive to heat.

Multiple myeloma: a clinicopathologic study of 62 consecutively autopsied cases.

The clinical, laboratory and pathologic findings were studied in 62 consecutively autopsied patients with multiple myeloma between 1954 and 1975. All patients were 40 years of age or older. Bone pain was the initial symptom in 2/3 of patients. Anemia (81%), thrombocytopenia (29%), azotemia (41%), hypercalcemia (46%) and hyperuricemia (52%), were common laboratory findings at diagnosis. Ninety-seven percent had a monoclonal protein in serum or urine. Extensive plasma cell replacement of marrow was invariably seen at autopsy although in 15% of patients no abnormality was found on skeletal survey. Extraskeletal spread (67%) was due to direct extension to paraosseous tissue resulting from cortical destruction and to distant organ involvement mainly of splenic red pulp and hepatic sinusoids. The patients were susceptible to bacterial infection, mainly gram-negative, of the lung (56%), urinary tract (35%), and blood (24%). Fungal infection was less frequent and usually consisted of superficial candidal overgrowth of gastrointestinal tract ulcerations (18%). Amyloidosis (10%) was perivascular and associated with light chain proteinuria. Renal failure as a cause of death (21%) was secondary only to infection (52%). Severity of histologic findings in the kidney at autopsy had little correlation to initial BUN concentration. The median survival was 11.5 months with alkylating agent therapy (responders, 29 months; non-responders, 6 months), and 6 months with urethan. Initial azotemia (greater than 80 mg/dl) and hypercalcemia (greater than 12 mg/dl) were important prognostic indicators (median survival, less than 1 month and 3 months, respectively). A good response to alkylating agent therapy, initial BUN less than 40 mg/dl and serum calcium less than 12 mg/dl were favorable to prognostic indicators.

Epstein-Barr virus-induced lymphoma in a cardiac transplant recipient.

A monoclonal diffuse histiocytic lymphoma developed during the course of a serologically documented primary Epstein-Barr virus infection in a 22-year-old cardiac transplant recipient taking cyclosporine and prednisone. Throat washings revealed the virus at tumor presentation, and the tumor was shown to contain Epstein-Barr nuclear antigen-positive cells and the viral genome. Prolonged inversion of the T cell helper/suppressor ratio was demonstrated. A brief course of acyclovir appeared to halt viral shedding in the throat but had no apparent effect on the tumor.

Melanotic neuroectodermal tumor of infancy. Clinicopathological, immunohistochemical, and flow cytometric study.

Twenty cases of melanotic neuroectodermal tumor of infancy (MNTI) are reported. The patients (13 females, seven males), whose ages ranged from 1 to 9 months (mean, 5 months), typically presented with a rapidly growing mass. Tumor sites included the maxilla (13 cases), mandible (three cases), dura (two cases), brain (one case), and skull/orbit (one case). The mean tumor size was 3.5 cm (range, 1.0-10.0 cm). Follow-up was obtained on 12 cases. Five tumors (45%) recurred within 4 months of diagnosis, but none metastasized. One surgical death occurred. Histologic appearance was distinctive, with tubular or alveolar formations of large melanin-containing cells around nests of smaller neuroblastic cells possessing scant or fibrillar cytoplasm. Twelve tumors were studied immunohistochemically; tumor was positive for cytokeratin in 12 of 12, for HMB 45 in 12 of 12, for vimentin in seven of eight, and for epithelial membrane antigen (EMA) in four of nine tumors, mainly in the large cells. Neuron-specific enolase (NSE) (seven of 12) and Leu 7 (nine of 12) were positive in small and large cells; some tumors also expressed synaptophysin (four of 12), glial fibrillary acidic protein (GFAP, three of 12 tumors), or S-100 protein (two of 12 tumors). No staining was found for chromogranin, desmin, or carcinoembryonic antigen (CEA). Eight of 10 tumors studied had interpretable results on flow cytometry (FCM) (four DNA diploid, three DNA aneuploid, and one DNA diploid with a prominent shoulder). Tumor recurred locally in two of five cases with follow-up, and we were unable to demonstrate the usefulness of FCM in predicting recurrences. Further studies are necessary to define better the potential usefulness of FCM in predicting aggressive behavior. Distinctive morphology and multiphenotypic (epithelial, neural, melanocytic) expression distinguish MNTI from melanoma and metastatic neuroblastoma.

Crystal-storing histiocytosis associated with lymphoplasmacytic neoplasms. Report of three cases mimicking adult rhabdomyoma.

Massive crystal deposition is rare in lymphoplasmacytic (LPc) or plasma cell neoplasms. We report three cases in which the accumulation of crystals in histiocytes closely reproduced the histologic features of adult rhabdomyoma. The patients, all female, aged 18, 77, and 78 years, presented with tumor of cervical lymph nodes (two cases) or the otolaryngic mucosa (two cases). In addition, two patients had monoclonal serum or urine immunoglobulin (IgM-kappa-1, unknown-1), and one had renal and bone marrow involvement on biopsy. This last patient died of acute renal failure at 5 months, another was alive without disease at 8 years, and the remaining one was lost to follow-up. Lymph nodes, mucosae, and kidney showed a neoplastic LPc infiltrate masked by sheets of large benign histiocytes containing sheaves of crystals. Paraffin-section immunohistochemistry demonstrated monoclonal staining of the LPc cells in all cases (IgM-kappa-2, IgA-kappa-1) and of the crystals (IgM-kappa) in one case. In all patients, the crystal-containing cells were positive for KP-1 (CD68), but not for desmin, muscle-specific actin, or myoglobin. These findings suggest that, in any case of adult rhabdomyoma in which the histologic findings are not typical, a crystal-storing histiocytosis should be ruled out: recognition of the atypical LPc component and the histiocytic immunophenotype of the crystal-storing cells will help prevent a serious misdiagnosis.

Hodgkin's disease of Waldeyer's ring. Clinical and histoimmunophenotypic findings and association with Epstein-Barr virus in 16 cases.

Waldeyer's ring is an uncommon, rarely reported primary site for Hodgkin's disease. We report a series of 16 such cases culled from the files of the Armed Forces Institute of Pathology and the National Cancer Institute. The patients' median age was 41 years (range, 14-74), and they presented with airway obstruction or unilateral tonsillar enlargement. The disease was localized to the Waldeyer's ring (stage I) in 46% of patients and extended to the cervical lymph nodes (stage II) in 39% and to the spleen (stage III) in 15%. Local radiation therapy, with or without chemotherapy, obtained a complete response in all but two patients. There was local recurrence in one patient and distant spread in three others. All patients for whom follow-up is available are alive without evidence of disease at 9 to 216 months (median, 20 months) except two who died of widespread Hodgkin's disease and two others who died of other causes. Histologically, eight cases were classified as mixed cellularity type (50%), four as nodular sclerosis (25%), and one as lymphocyte predominance, nodular (LPn; 6.3%); three others that showed interfollicular involvement were unclassified (18.7%). The Reed-Sternberg (RS) and atypical mononuclear cells in most cases of mixed cellularity and interfollicular types and all cases of nodular sclerosis had the classic immunophenotype (CD45-, CD20- and/or CD45RO-, CD15+ and/or CD30+). In the single case of LPn, they were of B-cell lineage (CD45+, CD20+, CD45RO-, CD15-, CD30-). In situ hybridization performed on routinely processed sections revealed Epstein-Barr virus (EBV) EBER1 mRNA in RS cells of eight of 12 cases studied (67%) only in mixed cellularity and nodular sclerosis, but not in LPn. We conclude that, however rarely, Hodgkin's disease of typical morphology and immunophenotype can originate in Waldeyer's ring. The incidence of EBV detection in the RS cells in our study is greater than that usually seen in nodal Hodgkin's disease in the United States. The greater prevalence of EBV-related Hodgkin's disease at this site is probably a reflection of the fact that the Waldeyer's ring is a reservoir for EBV.

Relationship of p53 gene alterations with tumor progression and recurrence in olfactory neuroblastoma.

Olfactory neuroblastoma (ONB) is a rare neuroectodermal tumor whose clinical course is not effectively predicted by initial stage or grade; p53 tumor suppressor gene alterations have not been determined concerning the ONB pathobiology and recurrence. We analyzed 18 formalin-fixed, paraffin-embedded ONB specimens (12 primary tumors and six recurrences or metastases) from 14 patients for p53 alterations using immunohistochemistry for p53 and WAF1 together with topographic genotyping (selection of minute tissue targets from unstained sections, PCR [polymerase chain reaction] amplification of exons 5-8 followed by direct DNA sequencing). Sequential material representing tumor recurrence or metastasis was available in four cases to compare genetic alterations over time in the same patient. None of the cases showed strong, diffuse p53 immunostaining. Focal weak to moderate intensity staining was evident in nine of 14 cases. Mutations in p53 were not detected in any of the cases, suggesting hyperexpression of p53 wild-type protein. Hyperexpression was further confirmed by correlation of WAF-1 and p53 immunopositivity. Importantly, in four cases with recurrence or metastasis, tumors manifested p53 wild-type hyperexpression. It appears that p53 point mutation does not play an important role in the initial development of ONB; however, p53 wild-type hyperexpression may occur in subsets of ONB likely to show local aggressive behavior and a tendency for recurrence. Wild-type p53 hyperexpression may be an important event in later stages of ONB growth and progression.

Immunolocalization of interleukin-6 in salivary gland tumors.

Interleukin-6 (IL-6) is a multifunctional cytokine that regulates immune responses and acute phase reactions. It has demonstrated a growth factor function in several tumors, including those of salivary, plasma cell, and renal origin. We performed immunohistochemical staining for IL-6 localization on 57 salivary tumors. Reactivity was scored by intensity (0 to 4+) and percentage of cells staining, and the tumors were classified into three groups representing low (0 to 1+, 0% to 30%), moderate (2 to 3+, 31% to 75%), or high (> 3 to 4+, 76% to 100%) reactors. High reactivity was found in all primary pleomorphic adenomas (N = 10), five of eight recurrent pleomorphic adenomas, and all polymorphous low grade adenocarcinomas (N = 4). Moderate reactivity was observed in four of seven basal cell adenomas and three of five myoepitheliomas. Low reactivity characterized all acinic cell carcinomas (N = 3) and mucoepidermoid carcinomas (N = 3) as well as six of nine primary adenoid cystic carcinomas and all metastatic adenoid cystic carcinomas (N = 3). Carcinoma ex pleomorphic adenoma (N = 5) had three low and two moderate reactors. A pattern emerged in which the benign and low grade malignant tumors showed stronger reactivity than the metastatic or high grade malignant tumors. This suggests an inverse relationship between the presence of IL-6 and the biological aggressiveness of salivary gland tumors. The function of IL-6 in salivary gland neoplasia awaits further study and elucidation.

Fetal rhabdomyoma of the head and neck: a clinicopathologic and immunophenotypic study of 24 cases.

Twenty-four fetal rhabdomyomas (FRMs) of the head and neck occurring in 16 male and seven female patients (sex unknown in one), ranging from 3 days to 58 years of age (median, 4.5 years) are reported. Ten patients (42%) were < or = 1 year old, six lesions (25%) were congenital, and 11 lesions (46%) occurred in patients > or = 15 years of age. The median tumor size was 3.0 cm (range, 1.0 to 12.5 cm). The FRMs presented as well-defined, solitary masses arising within the soft tissue or mucosa (2:1) of the head and neck. The median follow-up in 15 cases was 48 months (range, 2 months to 52 years) after diagnosis. With the exception of one patient with a local recurrence, all patients were either alive and well or dead of unrelated causes. Eight cases, regarded as "classic" FRM, consisted predominantly of bland, primitive spindled cells associated with delicate, elongated skeletal muscle cells reminiscent of fetal myotubules that were haphazardly arranged in an abundant fibromyxoid stroma. The remaining 16 cases, designated as "intermediate" FRM, displayed both a greater degree and a greater number of cells with skeletal muscle differentiation as well as a variety of distinctive cytologic and architectural features. These included the presence of large, ganglion cell-like rhabdomyoblasts with vesicular nuclei and prominent nucleoli, interlacing ribbon or strap-like rhabdomyoblasts with deeply acidophilic cytoplasm, broad bundles of more delicate spindled rhabdomyoblasts arranged in fascicles simulating smooth muscle, an occasional plexiform pattern with infiltration of adipose tissue and skeletal muscle, focal intimate association with peripheral nerves, and rare areas of fibroblastic proliferation. Mitoses were not found in 19 of the 24 FRM cases, but in five tumors there were 1 to 14 mitoses/50 high-power fields. Marked nuclear atypia, anaplasia, and a "cambium layer" were uniformly absent. The FRMs typically stained for myoglobin, desmin, and muscle-specific actin with focal or rare staining for vimentin, smooth muscle actin, S-100 protein, glial fibrillary acidic protein, and Leu-7. Cytokeratin, epithelial membrane antigen, and CD68 antigen (with KP1) were not detected. This study expands on previous reports of FRM and demonstrates that it has both a broader age range and histologic spectrum than previously recognized. The mitotic rates of FRM as well as certain histologic features overlap with rhabdomyosarcoma; the lack of marked nuclear atypia is an important distinguishing feature.(ABSTRACT TRUNCATED AT 400 WORDS)

Salivary gland lymphoid infiltrates associated with lymphoepithelial lesions: a clinicopathologic, immunophenotypic, and genotypic study.

The criteria for distinguishing benign lymphoepithelial lesions (BLEL) from low grade B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) type in salivary glands and the significance of genotypically documented clonality in this setting are controversial. In addition, the clinical implications of a neoplastic diagnosis are unclear. The histopathologic features of 68 specimens from 49 patients with at least one salivary gland biopsy with LEL together with available clinical data were, therefore, reviewed. Paraffin section immunohistochemical (IHC) stains for kappa, lambda, CD3, CD20, and CD43; in situ hybridization (ISH) for kappa and lambda; and polymerase chain reaction (PCR) for immunoglobulin (Ig) HC rearrangement were performed. The 61 salivary gland specimens were classified as BLEL-13, BLEL with monocytoid B-cell (MBC) halos (BLEL-halo-8), low grade B-cell lymphoma of MALT type with confluent zones of MBC or other atypical lymphocytes (ML-MALT-24), low grade B-cell lymphoma of MALT type with monoclonal plasma cells (ML-MALT-PC-12), and high grade B-cell lymphoma of MALT type (MALT-high grade-4). Soft tissue and perineural invasion was not observed in BLEL and was most common in the MALT lymphomas. Lymph node involvement was identified in six patients at the time of their salivary gland MALT lymphomas but in none with BLEL. CD43+ B cells were seen most commonly in ML-MALT but were present in all other categories except MALT-high grade. Clonal B cells were identified by PCR in 5 of 12 BLEL, 5 of 8 BLEL-halo, 17 of 22 ML-MALT, 6 of 10 ML-MALT-PC, and 3 of 3 MALT-high grade biopsies. All ML-MALT-PC were clonal by ISH or IHC. Repeat biopsies in 14 patients most commonly showed a BLEL/ML-MALT lesion in an ipsilateral or contralateral salivary gland with one transformation to a MALT-high grade. Although only a few patients are known to have received chemoradiation or radiation therapy, most patients with low-grade lesions have pursued an indolent course. These data show the presence of two types of borderline lesions within the spectrum of lymphoid proliferations associated with salivary gland LEL. One has clonal B cells without histological features of neoplasia and the other nonconfluent MBC extending beyond the confines of LEL ("halos"). They share some features with the infrequent nonneoplastic BLEL and others with the more common low-grade B-cell lymphomas of MALT. A few high-grade B-cell lymphomas of MALT were also identified including a rare example of transformation from a low- to high-grade lesion. The optimal therapeutic approach for the borderline and low-grade lesions and the reason why so many of the lymphoproliferative lesions associated with LEL remain localized to the neck remain to be defined.

Lymphoepithelial carcinoma of the larynx and hypopharynx: study of eight cases with relationship to Epstein-Barr virus and p53 gene alterations, and review of the literature.

Eight cases of lymphoepithelial carcinoma (LEC) of the larynx and hypopharynx were evaluated for clinicopathologic features, and the presence of the Epstein-Barr virus (EBV) and p53 alterations. The seven men and one woman, all of non-Asian descent, averaged 64 years of age. Eighty-eight percent had histologically confirmed cervical lymph node metastasis at diagnosis. None had systemic disease. Seven of eight patients available for follow-up (mean, 17.7 months) were alive and free of disease, although one did develop recurrent tumor in the neck. Four tumors were composed, histologically, of pure LEC. Four others had foci of both LEC and conventional squamous cell carcinoma. All eight tumors exhibited alterations in p53 expression, but none was positive for EBV. Combining these 8 cases with the 15 previously published cases in the English literature indicate that LEC in this site is a rare, rather aggressive tumor, primarily of older adults (mean, 62 years) with a propensity for early cervical lymph node metastasis and eventual distant dissemination and death from disease in about one third of patients. Although p53 alterations are common and of no apparent prognostic significance, LEC at this site seems to have little, if any, relationship to the EBV in patients of non-Asian origin.

The role of p53 mutation and protein expression in primary and recurrent adenoid cystic carcinoma.

Adenoid cystic carcinoma (ACC) is a malignant tumor of salivary gland origin having a propensity for spread by direct extension or perineural invasion with frequent recurrences. Previous reports have shown that tumor behavior is not always predicted by histological pattern or stage. Little is known of the role of p53 tumor suppressor gene mutation and altered protein expression with respect to ACC pathobiology and recurrence. The authors analyzed a group of 14 ACC specimens (seven primary; seven recurrent) from 13 patients treated between 1987 to 1993. Formalin-fixed, paraffin-embedded specimens were reviewed and subjected to, immunohistochemistry (p53, DO-7, DAKO, Nutley, NJ; and WAF-I, Ab-1, Oncogene Sciences, Uniondale, NY) on 4-microm-thick histological sections as a prelude to p53 genotyping. In one case, sequential material representing primary and recurrent tumor was analyzed. Each tumor specimen was topographically genotyped for p53 point mutational change. Minute tissue samples were removed from unstained sections, polymerase chain reaction (PCR) amplified for p53 exons 5 to 8, and then underwent direct DNA sequencing. Six of seven primary ACCs were p53 immunostain negative. Four of seven recurrent (57%) ACCs were p53 immunopositive. These tumors showed varying degrees of p53 immunopositivity ranging from diffuse, intense staining of most tumor cells (n = 1) to interspersed, strongly positive cells mixed with predominantly p53 immunonegative cells (n = 4). All tumors were WAF-I immunostain negative. Two of the most immunopositive recurrent tumors each manifested a single type of p53 point mutation detected by p53 DNA genotyping (p53 exon 5:codon 175 and p53 exon 6:codon 199). In the case in which both primary and recurrent tumor was available, only the recurrent tumor contained point mutational damage. Negative immunostaining for p53 in primary ACC suggests that p53 mutation is not important in early events involving development of this tumor. In contrast, the frequent presence of p53-positive cells and the detection of point mutations in recurrent ACC suggests that p53 alterations are involved in later stages of tumor progression, important in the phenomenon of ACC recurrence.

HIV-associated Waldeyer's ring lymphoid hyperplasias: characterization of multinucleated giant cells and the role of Epstein-Barr virus.

Lymphoid hyperplasia of Waldeyer's ring (WR) is an often-symptomatic complication of human immunodeficiency virus (HIV) infection. A characteristic but not well explained finding is the presence of multinucleated giant cells (MNGCs) adjacent to crypt or surface epithelium. To further elucidate the MNGCs and assess their relationship to HIV and Epstein-Barr virus (EBV), 12 specimens from 11 HIV-positive patients were stained with antibodies to HIV-1 p24, EBV (latent membrane protein, LMP-1), histiocytes (CD68), and other antigen-presenting cells: S-100 protein, the Langerhans cell (LC) marker CD1a, and the follicular dendritic cell (FDC) marker (CD21). Double immunofluorescent staining to assess co-expression of p24 and cell-specific markers was performed and analyzed by laser-scanning confocal microscopy with 3-dimensional reconstruction. In situ hybridization for EBV-encoded small RNA (EBER) was performed in all cases. Immunostains showed MNGCs labeled for p24, S-100, and CD68, but not CD1a. In 1 case, rare MNGCs were CD21-positive. EBV LMP-1 was uniformly negative, although EBER-positive lymphocytes were seen by in situ hybridization in 9 of 12 specimens (numerous in only 3 specimens). Double immunofluorescent staining showed co-localization of p24 with CD68 and S-100. Our results suggest that MNGCs are generally HIV-infected, EBV-negative, and most likely represent an unusual S-100-positive histiocyte subset (not LC or FDC). Their exact pathophysiologic role remains uncertain. EBV does not appear to play a major role in the pathogenesis of WR lymphoid hyperplasias in HIV infection.

Extranodal sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of the head and neck.

We report 14 cases of extranodal sinus histiocytosis with massive lymphadenopathy involving a variety of head and neck sites. The patients ranged in age from 3 to 70 years (median, 43 years). Nine cases occurred in women and five occurred in men. The clinical presentation varied depending on the site of occurrence and included nasal obstruction, stridor, proptosis, ptosis, decreased visual acuity, facial pain or tenderness, cranial nerve deficits, mandibular tenderness, and mass lesions. Head and neck sites involved by disease included the nasal cavity, paranasal sinuses, nasopharynx, parotid gland, submandibular gland, larynx, temporal bone, infratemporal fossa, pterygoid fossa, meninges, and orbital region. The majority of patients presented with involvement of more than one site. Nodal involvement was identified in four patients. Special stains for microorganisms were negative. The sinus histiocytosis with massive lymphadenopathy cells demonstrated an immunophenotypic profile supporting derivation from macrophage/histiocytic lineage. Treatment varied and included surgical excision with or without adjuvant therapy (chemotherapy, radiotherapy) or steroids. Several patients required more extensive surgery as a result of extension of their disease to adjacent structures or due to recurrent disease. Twelve patients are alive and either free of disease or have persistent disease. Two patients died, one as a result of complications of disease.

Adult rhabdomyoma of the head and neck: a clinicopathologic and immunophenotypic study.

Twenty-seven cases of adult rhabdomyoma (ARM) of the head and neck are reported. The 20 male and seven female patients ranged in age from 33 to 80 years (median age, 60 years). Symptoms included airway obstruction and a mass within the mucosa or soft tissue. Median tumor size was 3.0 cm (range, 1.5 to 7.5 cm). Seven patients (26%) presented with multinodular tumors and one tumor was multicentric. Follow-up was available in 19 cases and ranged from 2 months to 18.5 years after diagnosis (median, 6.0 years). Lesions recurred locally in eight cases (42%) 2 to 11 years after diagnosis (median, 6 years). One recurrence was multicentric. Histologically, ARM was composed of closely packed, large polygonal cells having abundant, eosinophilic, granular, or vacuolated glycogen-rich cytoplasm with focal cross-striations. Immunohistochemical stains confirmed skeletal muscle differentiation; the majority of tumors stained for myoglobin (21 of 21 tumors), muscle-specific actin (21 of 21 tumors), and desmin (19 of 21 tumors). Focal or rare immunoreactivity for vimentin (six of 17 cases), alpha-smooth muscle actin (17 of 20 cases), S-100 protein (14 of 21 cases), and Leu-7 (10 of 20 cases) also was detected. Cytokeratin, epithelial membrane antigen, glial fibrillary acidic protein, and CD68 antigen (with KP1) were not found. The characteristic histology and immunophenotype distinguish ARM from other lesions with which it is frequently confused, including granular cell tumor, hibernoma, oncocytoma, and paraganglioma. The expression of alpha-smooth muscle actin has not been reported previously in ARM; its presence could reflect aberrant expression of smooth muscle actin in skeletal muscle or possibly be a recapitulation of early skeletal muscle embryogenesis.

Acute myelogenous leukemia following immunosuppressive therapy for rheumatoid arthritis.

A 51-year-old woman had acute myelogenous leukemia following log-term cyclophosphamide therapy for rheumatoid arthritis. After standard methods of management had failed, the rheumatoid arthritis showed considerable improvement in response to approximately 25 mg cyclophosphamide per day over a four-year period. At the end of this period, pancytopenia developed, and cyclophosphamide was discontinued. A bone-marrow aspirate showed nonspecific changes. However, four months later because of severe progressive pancytopenia, another bone-marrow examination was performed; it showed acute myelogenous leukemia. Therapy failed to induce a remission, and two months after diagnosis the patient died of aspergillosis. Autopsy confirmed the presence of leukemic infiltration of bone marrow, lymph nodes, liver and spleen. These findings suggest a possible leukemogenic role of cyclophosphamide in this case and suggest that caution should be exercised in treating non-fatal diseases with antitumor drugs.

Tumor-simulating retrorectal heterotopia of bone marrow.

A retrorectal tumor-simulating mass of extramedullary hematopoiesis was discovered during work-up for uterine bleeding in a 40-year-old woman. It was excised in toto. The only hematologic abnormality at the time was iron deficiency, which was corrected. Six years later the patient is free of recurrence or any hematologic abnormality. It is suggested that this case represents heterotopic marrow arising either as a remnant of embryonic hematopoiesis or from activated primitive cells retaining the embryonic potentiality of hematopoiesis. The difficult diagnostic problems, pathogenesis and treatment are discussed, and the importance of recognizing the trilineage of hematopoiesis using Wright's-stained imprints of the mass is emphasized.

Primary lymphadenopathic Kaposi's sarcoma in an immunocompetent 23-year-old man.

An aggressive visceral form of Kaposi's sarcoma (KS) has been reported in immunosuppressed populations, most notably in patients with acquired immunodeficiency syndrome (AIDS). This presentation contrasts from the indolent skin lesions on the extremities of older males and is distinct enough in the young adult American population to have been included in the working definition of AIDS. The authors describe a case of primary lymphadenopathic KS in an immunocompetent heterosexual 23-year-old man with no epidemiologic, immunologic, or serologic evidence of AIDS. This report emphasizes that a diagnosis of KS in a young American male is not synonymous with the diagnosis of AIDS.

Postmenopausal ureteral endometriosis with atypical adenomatous hyperplasia following hysterectomy, bilateral oophorectomy, and long-term estrogen therapy.

Endometriosis of the ureter is rare. The authors describe a case of ureteral endometriosis presumed on clinical and radiologic grounds to be a primary ureteral malignancy. The unique feature of the present case is the development, after long-term estrogen therapy, of postmenopausal ureteral endometriosis with extensive atypical adenomatous hyperplasia. The present case demonstrates that reactivation of endometriosis and the development of atypical hyperplasia may occur after menopause with prolonged estrogen therapy.

A near-haploid bone marrow karyotype in systemic mast cell disease: is it characteristic of the disease or an incidental finding?

We present the case of a 40-year-old man with aggressive systemic mast cell disease. The patient had a predominant near-haploid clone in his bone marrow cells, detected by cytogenetic analysis performed at the time of diagnosis. The similarities between this case and a previously published case of near-haploidy in a patient with malignant mastocytosis suggest that near-haploidy may be a characteristic of aggressive systemic mast cell disease rather than an incidental finding.