Littoral cell angioma of the spleen: an additional report of four cases with emphasis on the association with visceral organ cancers.

AIMS AND BACKGROUND: Littoral cell angioma (LCA) is an uncommon vascular tumor of the spleen recently described and interpreted as the tumoral counterpart of the normally present littoral cells lining the splenic sinus channels of red pulp. The diagnosis of LCA is suggested by a quite characteristic morphology and confirmed by the demonstration of a hybrid endothelial/histiocytic phenotype. METHODS: Four original and previously unreported cases of LCA are presented. All four splenic vascular tumors were investigated by light microscopy and immunohistochemistry for endothelial and histiocytic markers. RESULTS: All four cases were associated with visceral epithelial malignancies (colorectal adenocarcinoma in two cases, renal and pancreatic adenocarcinoma in one case each). One case was also associated with an intracranial tentorial meningioma. CONCLUSIONS: We consider our findings as a novelty and signal the possible existence of a clinical syndrome. Five of a total of 21 previously reported cases in the literature were also described as being associated with other cancers (non-Hodgkin's lymphoma in two cases, two not further specified tumors of the liver and brain, an epithelial ovarian cancer, and a non-small cell lung cancer in one case each). Close follow-up and careful investigation in search of a second visceral neoplasm are strongly recommended in cases of LCA, but further clinical observations and more in-depth genetic and molecular studies are needed before any valid conclusions can be drawn.

CD30-positive anaplastic large cell lymphoma (ALCL) of T-cell lineage in a 14-month-old infant with perinatally acquired HIV-1 infection.

PURPOSE: CD30-positive anaplastic large cell lymphoma (ALCL) has been described in adults with HIV-1 infection but is extremely rare in HIV-1-infected infants and children. PATIENT AND METHODS: A 14-month-old girl with congenitally acquired HIV-1 infection presented with fever and a tender, erythematous, cystic mass on the right labium majorum. The mass was biopsied. Histologic examination and immunohistochemistry were performed. RESULTS: Histologic examination showed Touton-like giant cells resembling histiocytes and focally abundant neutrophils obscuring the lymphoid infiltrate. Immunocytochemistry revealed a CD30-positive ALCL of T-cell lineage. CONCLUSION: Although non-Hodgkin's lymphoma is known to be associated with HIV-1 infection in children, large cell lymphomas of T-cell lineage are extremely rare in this population. Early diagnosis should be aggressively pursued in an HIV-1-infected child who presents with a fever and cutaneous mass.

Cytologic features of deep juvenile xanthogranuloma.

Juvenile xanthogranuloma (JXG) is a rare lesion of the skin and deep tissues whose cytologic features have not previously been published. We report on 2 cases of JXG in patients aged 5 days and 5 yr who presented with an axillary mass and enlarged supraclavicular lymph node, respectively. Fine-needle aspiration and touch imprints made from the surgical biopsies yielded cellular specimens composed of histiocytes, multinucleated Touton-type giant cells, and variable admixtures of lymphocytes and eosinophils. One lesion was composed primarily of benign-appearing histiocytes with admixed multinucleated giant cells and a few lymphocytes. The second lesion consisted of a few histiocytes, occasional Touton giant cells, many lymphocytes, and scattered fibroblasts. The histiocytes were CD68-positive and S100-negative. Histologic follow-up confirmed the diagnosis of juvenile xanthogranuloma. One patient was lost to follow-up; the other is alive without evidence of recurrence 1 yr after surgery. We conclude that deep JXG has characteristic and diagnostic cytologic features.

Granular histiocytosis of pelvic lymph nodes following total hip arthroplasty. The presence of wear debris, cytokine production, and immunologically activated macrophages.

UNLABELLED: Infiltration of regional lymph nodes by macrophages has been demonstrated after total joint arthroplasty. Although lymph nodes regulate the immune response, neither cytokine production nor the degree of immunological activation of cells within these nodes after total joint arthroplasty has been investigated. Pelvic lymph nodes were obtained from five patients who had had a total of eleven arthroplasties in seven hips three to twenty years before a pelvic staging procedure for adenocarcinoma (of the prostate in four patients and of the endometrium in one). All lymph nodes had polyethylene or metal debris as well as effacement of the normal nodal architecture by a histiocytic infiltrate. These changes were bilateral in the patients who had had an arthroplasty of one hip. Analysis of specimens from pelvic lymph nodes on the side of the involved hip demonstrated intense immunohistochemical staining of histiocytes for the major histocompatibility complex class-II antigen HLA-DR, a marker of histiocyte immune activation. In contrast, staining was absent in specimens from the contralateral lymph nodes as well as in those from seven patients who had had a prostatectomy but not a hip arthroplasty. Immunohistochemical staining for interleukin-1beta, tumor necrosis factor-alpha, and interleukin-6 demonstrated a much greater expression of these cytokines in the involved lymph nodes. CLINICAL RELEVANCE: Additions improvements in total joint replacement will be facilitated by a more thorough understanding of the biological response to the components and materials of implants. While local biological factors leading to failure of prostheses are currently under intense investigation, the mechanisms and importance of regional and systemic immune responses to wear debris require further study.

Extrapelvic endometriosis: diagnosis and treatment.

BACKGROUND: Young women with nondescript abdominal pain can be difficult to diagnose. Although extrapelvic endometriosis is infrequent, we have treated 7 patients over the past 3 years with endometriosis in the abdominal wall, inguinal canal, or surgical incisions as the etiology of their symptoms. PATIENTS AND METHODS: We reviewed the medical records of patients whose final pathology report confirmed a diagnosis of extrapelvic endometriosis. Seven women who were treated at the University of Rochester Strong Memorial Hospital from May 1, 1991 through April 30, 1994 were identified. RESULTS: All patients were premenopausal with no history of pelvic endometriosis. In 4 patients, symptoms were cyclical. Surgical excision was initially curative in 5 patients. Two women required reexcision. The diagnosis of endometriosis was established at exploration by gross appearance and by frozen section. CONCLUSIONS: Endometriosis should be included in the differential diagnosis of a symptomatic mass in a celiotomy scar, the abdominal wall, or the inguinal canal. Principles of management include obtaining an accurate diagnosis and performing an adequate excision to prevent recurrence.

Solitary fibrous tumors of soft tissue. A clinicopathologic and immunohistochemical study of 12 cases.

We describe 12 cases of primary soft tissue neoplasms that showed the histologic and immunohistochemical features of solitary fibrous tumors of serosal surfaces (solitary fibrous mesothelioma, submesothelial fibroma). Nine patients were women and three were men whose ages ranged from 28 to 83 years. Seven lesions were located in the head and neck region, and the remainder were located in the back, buttock, perineum, and groin. The lesions measured from 1 to 6 cm in greatest diameter and presented grossly as well-circumscribed, unencapsulated, soft to rubbery tissue masses. Histologically they were characterized by a proliferation of spindle cells exhibiting a variety of growth patterns, including storiform, herringbone, neural with wavy nuclei, and hemangiopericytic admixed with areas of sclerosis. In two cases the lesions showed areas of increased cellularity with occasionally scattered mitotic figures. Three cases were located adjacent to a major salivary gland; in one, entrapment of normal salivary gland acini and ducts could be observed at the edges of the lesion. Immunohistochemical studies showed positive staining of the spindle cells with CD34 (anti-HPCA-1) and vimentin antibodies and negative staining with keratin, actin, desmin, S-100 protein, collagen type IV, and factor VIII related antigen. Follow-up from 6 months to 12 years has shown no evidence of recurrence or metastasis in any of our patients. Solitary fibrous tumors appear to represent ubiquitous mesenchymal neoplasms that may not be necessarily restricted to serosal surfaces. Identification of these lesions is of importance to avoid misdiagnosis with other more aggressive conditions in soft tissue locations.

Analysis of the effect of cytokines (interleukins 2, 3, 4, and 6, granulocyte-monocyte colony-stimulating factor, and interferon-gamma) on generation of primary cytotoxic T lymphocytes against a weakly immunogenic tumor.

We have investigated the role of cytokines (IL-2, IL-3, IL-4, IL-6, IFN-gamma, and GM-CSF) in the generation of primary cytotoxic T lymphocytes (CTL), within a single tumor system. The murine carcinoma line 1 was transfected with expression vectors with cDNA for these cytokines. Line 1 expresses low levels of class I MHC molecules, but can be induced with dimethyl sulfoxide or IFN-gamma to express high levels of class I. Class I low line 1 cells are not susceptible to CTL lysis, while class I high line 1 are lysed by CTL, which allows us to assay for CTL activity. To isolate primary CTL, tumor-infiltrating lymphocytes were isolated from cytokine-expressing or control tumors, growing in vivo. Most cytokines stimulated nonspecific killers, but IL-2 and IL-3 stimulated primary CTL. While IFN-gamma alone did not generate primary CTL, coexpression of IFN-gamma with IL-2 resulted in CTL generation. This is the first comparison of the effects of a series of cytokines on primary CTL development, and has important implications for vaccine development and immunotherapy.

Surgical pathology of the uterine cervix. Diagnostic problems and controversies.

Some of the more interesting and controversial issues concerning the pathology of the uterine cervix have been addressed in this article. It is hoped that this brief overview will serve as a stimulus for the reader to delve into some of these topics in greater detail either by critical examination of the literature or reporting one's own experience with these unusual tumors. A heightened awareness of the aforementioned "tumor-like" lesions may avert a misdiagnosis of malignancy with potentially disastrous consequences.

The acute respiratory distress syndrome: current trends in pathogenesis and management.

Management of patients in ARD remains a complex diagnostic and difficult therapeutic problem. Although the authors have focused on several new and exciting management strategies, it is important to recognize that the vast majority of patients with ARDS simply require meticulous general medical care. In this era of invasive monitoring and other care. In this era of invasive monitoring and other complex technologies, there is sometimes a tendency to neglect the basic issues such as judicious use of antibiotics to avoid superinfection with resistant organisms or antibiotic-associated diarrhea; removal of unnecessary intravenous lines to minimize line related infections; and maintenance of adequate nutrition to promote good skin and mucosal integrity as well as preserving important muscle mass all too often receive a low priority. It is anticipated that with meticulous general medical care, as well as selective implementation of the above treatment strategies, improvement in the outcome of patients is an achievable goal.

Hepatic cysticercosis. Report of an unusual case.

We report a case of hepatic cysticercosis in a 62-year-old man who had been diagnosed with a colonic adenocarcinoma 9 years previously. Additional clinical studies failed to show disease in the soft tissues, brain, orbit and heart. The patient was successfully treated with a course of praziquantel and prednisone and is alive and well 28 months after diagnosis. This is the first reported case of hepatic cysticercosis that has resulted in significant morbidity.

Primary lymphoma of bone. Correlation of magnetic resonance imaging features with cytokine production by tumor cells.

BACKGROUND: Primary lymphoma of bone is a rare, aggressive neoplasm that can present with a large, soft-tissue mass despite minimal evidence of cortical destruction on plain radiographs. METHODS: High resolution magnetic resonance imaging (MRI) examinations of four patients with primary lymphoma of bone were reviewed retrospectively, and in each case intramedullary tumors demonstrated "penetrating channels" extending through the cortex. The MRI studies were correlated with the histopathologic assessment of the tumor for each patient. Immunohistochemistry was performed for immunophenotyping and for cytokine expression by tumor cells. The cytokines that were investigated were interleukin-1, interleukin-6, and tumor necrosis factor-alpha, molecules known to regulate osteoclastic activity. RESULTS: The linear cortical foci noted on MRI correlated with the histopathologic findings of tumor-associated cutting cones, in proximity to osteoclastic bone resorption. Immunohistochemical stains showed a B-cell phenotype for each tumor and positive immunoreactivity in tumor cells for cytokine mediators that stimulate osteoclastic activation. CONCLUSIONS: These findings indicate that the tumor cells in these cases produce soluble cytokine mediators that may regulate extensive osteoclastic activity. In primary lymphoma of bone, tumor activation of osteoclastic resorption, with production of tumor tunnels through the cortex, may represent one of the mechanisms by which lymphoma escapes the intramedullary space and forms large, soft-tissue masses without extensive cortical destruction.

Placental metastases in a patient with recurrent breast carcinoma.

We present an unusual case of extensive placental micrometastases of recurrent breast carcinoma. The discrepancy between the gross and histopathologic findings in this case suggests that in the absence of gross metastases, meticulous microscopic examination may reveal a higher incidence of metastases than has been reported previously.

Anomalous immunostaining of 'optically clear' nuclei in gestational endometrium. A potential pitfall in the diagnosis of pregnancy-related herpesvirus infection.

A distinctive morphologic alteration originally described as nuclear "clearing" has been observed in endometrial epithelium during pregnancy. Affected nuclei have a clear center and a rim of marginated chromatin, imparting a "glassy" appearance reminiscent of herpesvirus inclusions. We observed this change in gestational endometrium attached to the membranes of an otherwise normal-term placenta. Because of the morphologic suggestion of herpesvirus infection, immunoperoxidase studies using the avidin-biotin method were performed, resulting in intense staining of nuclear inclusions. An identical pattern of nuclear staining was also noted in negative controls. This anomalous staining reaction was replicated with the streptavidin-biotin method but not with the peroxidase-antiperoxidase method. Furthermore, nuclear staining was abolished after sequential preincubation with free avidin (0.05%) and free biotin (0.05%). Using the peroxidase-antiperoxidase method, positive nuclear staining was identified with monoclonal antibodies to biotin. Similar results were obtained in nine additional cases of optically clear nuclei associated with term placentas. We conclude that staining of optically clear nuclei is the result of avidin and streptavidin binding to intranuclear biotin. This anomalous staining pattern could potentially result in the misdiagnosis of herpesvirus infection.

In utero adrenal hemorrhage associated with fetal distress, subsequent transient neonatal hypertension, and a nonfunctioning ipsilateral kidney.

We present an unusual case of in utero adrenal hemorrhage associated with fetal distress. This case was further complicated by transient neonatal hypertension that resolved spontaneously and a nonfunctioning ipsilateral kidney. We discuss the possible underlying pathophysiology.

Cutaneous ciliated cyst of the scalp. A case report with immunohistochemical evidence for estrogen and progesterone receptors.

The cutaneous ciliated cyst (CCC) is an unusual lesion found in young women that typically occurs on the lower extremities. This report describes a case of CCC arising in the scalp, a previously undescribed anatomic location for this lesion. In addition, positive nuclear staining for estrogen and progesterone receptors is demonstrated by immunohistochemical methods. Possible pathogenetic mechanisms are discussed.

Differential diagnosis of fat-containing lesions with abdominal and pelvic CT.

Recognition of fat within an organ or lesion on abdominal and pelvic computed tomographic scans is an important clue to guiding a differential diagnosis. A systematic approach to these lesions, including a patient's age and clinical history, along with the appearance and location of the lesion often allows a specific differential diagnosis. The anatomic sites of origin for these lesions are the gastrointestinal tract, genitourinary system, and retroperitoneum. Some of the more common entities include various forms of fatty change in the liver, fibrofatty mesenteric proliferation in Crohn disease, ovarian dermoids, and herniations of abdominal fat. In addition, pitfalls such as pathologic processes engulfing normal fat (eg, perirenal abscess) and iatrogenic incorporation of normal fat (eg, omental packing in liver lacerations) should also be included in the differential diagnosis. Familiarity with certain benign occurrences, such as focal fat in the liver adjacent to the falciform ligament and lipomatous infiltration of the ileocecal valve, obviates invasive diagnostic procedures.

Meigs syndrome with elevated serum CA 125.

Elevation of serum CA 125 levels is associated with malignant serous ovarian carcinomas and has been described in one patient with Meigs syndrome. We present two additional cases of Meigs syndrome with significant elevations in serum CA 125 and report the results of immunohistochemical staining for CA 125. CA 125 expression was localized to the omentum and peritoneal surfaces rather than the fibroma. We suggest that expression of CA 125 and accumulation of ascites in these two patients may have resulted from some common peritoneal process.