Granular cell tumor of the biliary tree. A report of two cases and a review of the literature.

Granular cell tumors are relatively uncommon soft tissue tumors usually presenting in the skin and subcutaneous tissues or tongue, although many sites have been described. Two cases arising in the extrahepatic biliary tree are described, and the previously reported cases of this rare presentation are reviewed. These tumors may mimic sclerosing cholangitis and cholangiocarcinoma clinically, and occasionally histologically, in this relatively young group of patients. The histogenesis appears to be related to Schwann cells as in granular cell tumors of other sites, evidenced by histologic, immunohistochemical, and electron microscopic findings. Granular cell tumors, albeit rare, should be considered in the differential diagnosis of biliary tract disease in young patients, particularly black women, and are curable by surgical excision.

The morphologic features of diversion colitis: studies of a pediatric population with no other disease of the intestinal mucosa.

Studies of diversion colitis have not shown a consistent pattern of histopathologic features, and many descriptions are difficult to interpret because of the presence of underlying intestinal mucosal disease. To define the histologic changes in patients free of other mucosal inflammatory disease, we studied the resected segments of bypassed colorectum from 37 patients with Hirschsprung's disease treated by a two-stage procedure, using rectal biopsy specimens taken for initial diagnosis and trimmings from proximal to the stoma as controls. Biopsy specimens from a further 14 patients of similar age but without colorectal mucosal disease were used as additional controls. The histology of the bypassed segment was abnormal in all patients. Twenty-six had diversion colitis characterized by diffuse follicular lymphoid hyperplasia; lamina propria expansion by plasma cells, lymphocytes, and some neutrophils; cryptitis; reactive epithelium; and mucin depletion. Crypt abscesses, aphthous ulcers, mild architectural distortion, and Paneth cell metaplasia were noted in more severe cases. The remaining 11 patients had mild follicular lymphoid hyperplasia and an increase in lymphoplasmacytic infiltrates, with absence of neutrophils, epithelial injury, and other changes seen in diversion colitis, a pattern we term "diversion reaction." Diversion colitis is common in children with a bypassed colorectum. It can be distinguished histologically from other mucosal diseases in most cases. We hypothesize that diversion reaction may be an inevitable consequence of colonocyte nutrient deficiency and that diversion colitis may be superimposed by a second insult, such as a low-grade pathogen.

Improved ultrastructural lung preservation with prostaglandin E1 as donor pretreatment in a primate model of heart-lung transplantation.

Donor pretreatment with prostaglandin E1 as a pulmonary vasodilator has developed as a simple, effective means to provide excellent preservation in heart-lung transplantation. This study was undertaken to investigate the degree of ultrastructural preservation of the lung with prostaglandin E1 and other pulmonary vasodilators in a primate heart-lung transplantation model. Heart-lung transplantation was performed in 14 African green monkeys. Donor cardiac preservation was achieved with cold crystalloid cardioplegic solution (10 ml/kg). Lung preservation was achieved with cold, modified Euro-Collins solution delivered into the main pulmonary artery (60 ml/kg total). Vasodilator agents were administered intravenously 15 minutes before aortic crossclamping. The heart-lung grafts were stored at 4 degrees C for 6 hours. Three groups of animals were studied: five donors with prostaglandin E1 (0.1 to 4.0 micrograms/kg per minute), five donors with prostacyclin (0.1 to 0.35 micrograms/kg per minute), and four donors with nitroprusside (0.8 to 5.0 micrograms/kg per minute). After transplantation, arterial blood gas measurements and lung biopsies were performed at 1- and 3-hour intervals. Five formalin blocks per specimen were sectioned for hematoxylin and eosin staining. Cellular preservation and endothelial cell swelling were evaluated with electron microscopy. The specimens were graded for alveolar hemorrhage, endothelial cell swelling, and cellular preservation (grade 0, minimal, to grade 3, severe) and a mean score was obtained for each preservative agent. Prostaglandin E1-treated specimens demonstrated the least amount of endothelial swelling (mean score of 1.0) compared with prostacyclin- and nitroprusside-treated specimens (mean scores of 1.4 and 2.7, respectively). All nitroprusside-treated specimens demonstrated moderate to severe endothelial cell swelling. Interstitial and alveolar hemorrhage was noted in poorly preserved specimens, but there were no significant differences between groups. We conclude that prostaglandin E1 provides improved cellular preservation by decreasing the extent of endothelial cell swelling as observed on electron microscopy.

Unilateral cutaneous emboli of Aspergillus.

A 40-year-old white woman with acute nonlymphocytic leukemia, which relapsed despite bone marrow transplantation and various chemotherapeutic regimens, developed fever and neutropenia. Her fever was unresponsive to broad-spectrum antibiotics, and on hospital day 53 she developed purpuric macules with necrotic centers on her left hand and forearm. Frozen sections of lesional skin were stained with Grocott's methenamine-silver and showed hyphae consistent with a species of Aspergillus; culture of the skin biopsy specimen yielded a pure culture of Aspergillus flavus. Localization of the emboli to the left upper extremity was subsequently explained by magnetic resonance imaging scan of the chest demonstrating invasion of the left subclavian artery by a pulmonary aspergilloma.

Lymphomatoid granulomatosis of the skin and lung. An angiocentric T-cell-rich B-cell lymphoproliferative disorder.

OBJECTIVE: To test the recent hypothesis that lymphomatoid granulomatosis (LYG) is a clonal B-cell proliferative process related to Epstein-Barr virus (EBV). BACKGROUND AND DESIGN: Historically, LYG has been classified as an angiocentric T-cell lymphoproliferative disorder. To further characterize LYG in the skin, we analyzed for EBV RNA in lymphocytes using in situ hybridization, coupled with colabeling for B-cell and T-cell markers. Clonality of lymphocytes was assessed by polymerase chain reaction using primers for immunoglobulin heavy chain genes and T-cell receptor beta and gamma genes. SETTING: Academic referral center. PATIENTS: In a 5-year retrospective review, we identified 4 patients with classic clinical and pathologic features of LYG in skin and lung, and tissue available from both sites. MAIN OUTCOME MEASURES: The presence or absence of EBV RNA and clonal gene rearrangements in cutaneous and pulmonary lesions of LYG. RESULTS: Biopsy specimens of skin and lung in all patients revealed angiocentric infiltrates predominantly composed of T lymphocytes. Epstein-Barr virus RNA was identified in the skin of 1 patient and the lung of 3 patients, and was restricted to B cells. Polymerase chain reaction revealed clonal immunoglobulin heavy chain gene rearrangements and no clonal rearrangement of T-cell receptor genes in skin and lung tissue of all patients. CONCLUSIONS: At least some examples of LYG in the skin and lung are characterized by a clonal proliferation of B lymphocytes, some of which contain EBV RNA. The B cells are typically scarce and may be obscured by striking angiocentric T-cell infiltrates.

The fate of the skin graft in laryngoplasty.

In laryngoplasty procedures, laryngotracheal soft tissue defects are often repaired using skin grafts. While stenting is necessary to approximate and immobilize the graft, prolonged stenting causes increased bacterial counts, granulation tissue formation, tissue ischemia, and graft failure. Optimal time for stent removal has not been experimentally defined. Using the ferret animal model, 24 laryngoplasty procedures were performed. The subjects were stented by group for 0, 3, 7, 14, or 28 days. Analysis consisted of quantitative bacteriology, dye perfusion, and quantitative histologic assessment of graft viability. Tissue culture results revealed that by 3 days after the procedure all groups had 10(5) CFU of bacteria per gram of tissue. Graft viability in successful procedures was maximal in the 7-day group and statistically significant from the 3-day to the 28-day groups. In conclusion, while stenting is necessary for graft adherence, prolonged exposure to local tissue sepsis leads to progressive graft destruction.

Squamous cell carcinoma arising in a thyroglossal duct cyst.

Carcinoma arising in a thyroglossal duct cyst is a rare event, occurring in less than 1% of abnormalities of the thyroglossal duct. To date, there have been approximately 100 cases reported, with papillary carcinoma accounting for the vast majority. Squamous cell carcinoma, on the other hand, is an even rarer event. Its clinical course in the elderly, consisting of recurrent drainage and suppuration, may distinguish it from other neoplastic conditions of thyroglossal duct cyst. We present a 65-year-old man with squamous cell carcinoma in a recurrently discharging thyroglossal duct cyst to illustrate important distinguishing clinical features of this condition.

Heterotopic bone formation in the gastrointestinal tract.

Heterotopic bone is found rarely in the gastrointestinal tract. Here we report four cases, one of which occurred in Barrett's esophagus in an area of metaplasia of specialized cell type without evidence of neoplasia. In the remaining cases, bone was associated with mucin-producing tumors of the appendix, transverse colon, and rectum. The clinical and pathologic findings are discussed in detail. Heterotopic bone formation in the digestive tract usually occurs in the colorectum in association with benign or malignant epithelial tumors, and most often with those that produce abundant mucin. The pathogenesis of the osseous metaplasia in the gastrointestinal tract is not understood, although mucin extravasation is an almost constant feature. Fragments of metaplastic bone in a biopsy of a mucinous neoplasm of the digestive tract should not be misinterpreted as osseous invasion.

Immunophenotypic characterization of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease).

Histochemical and immunohistochemical studies have been reported in only a few cases of sinus histiocytosis with massive lymphadenopathy (SHML) to date. These indicate that SHML cells belong to the macrophage/histiocyte family, but their exact origin is still unknown. We determined the antigenic phenotype of SHML cells in sections from 20 cases of routinely fixed, paraffin-embedded tissue and from two cases of fresh frozen tissue using a broad panel of antibodies to macrophage/histocyte, B-, and T-cell antigens. SHML cells expressed the following: (1) S-100 protein, (2) "pan-macrophage" antigens such as EBM11, HAM 56, and Leu-M3, (3) antigens functionally associated with phagocytosis (Fc receptor for IgG, complement receptor 3), and lysosomal activity (lysozyme, alpha 1-antichymotrypsin, and alpha 1-antitrypsyn), (4) antigens associated with early inflammation (Mac-387, 27E10), (5) antigens commonly found on monocytes, but not tissue macrophages (OKM5, Leu-M1), and (6) "activation" antigens (Ki-1 and receptors for transferrin and interleukin 2). These data suggest that SHML cells are true functionally activated macrophages that may be recently derived from circulating monocytes.

The effects of supercooling chemicals on myocardial ultrastructure: a transmission electron microscopy case study.

Extended ischemia results in organ infarction which limits the availability of donor hearts. Hypothermic storage extends heart preservation by effectively stopping cellular metabolism, thereby preventing toxic accumulations of metabolic wastes and depletion of energy stores. However, cell swelling as a result of ion concentration changes and cell laceration due to ice crystal growth are consequences of hypothermic ischemia. Supercooling successfully preserves hearts for an extended time without associated myocardial necrosis. The efficacies of four supercooling preservative solutions, containing hypertonic glucose, polyethylene glycol, and or winter flounder antifreeze protein, are assessed using the Langendorff isolated organ perfusion apparatus and transmission electron microscopy. Polyethylene glycol seems the most effective in preventing myocardial necrosis possibly by dehydrating, minimizing cellular ice formation, protecting against cell swelling, and functioning as an antioxidant. Hypertonic glucose seems the most effective in reducing cell swelling; it may also depress solution freezing points, bind water, adjust both intra- and extracellular osmolarities, stabilize proteins, and assist in adenosine triphosphate (ATP) production. Antifreeze protein seems to bind effectively to ice and inhibit its growth; it may also reduce membrane permeabilities to Ca2+ and K+ ions.

Infantile hemangioendothelioma of the pelvis associated with Kasabach-Merritt syndrome.

A case of infantile hemangioendothelioma of the pelvis in a newborn male infant is described. Shortly after delivery, a large abdominal mass was found by external examination. CT scan revealed a hypervascular retroperitoneal pelvic mass invading the lumbar spinal column. Exploratory laparotomy was performed and biopsy revealed infantile hemangioendothelioma. The tumor was associated with profound thrombocytopenia, intratumoral hemorrhage, and right hydroureteronephrosis. After treatment with high dose steroids, Cytoxan, and external beam radiation for 2 months, the patient was discharged with persistent thrombocytopenia requiring platelet transfusions. The hydroureteronephrosis has also not improved. The literature on this subject is reviewed.

Metastatic dermatofibrosarcoma protuberans with fibrosarcomatous change in the absence of local recurrence. A case report of simultaneous occurrence with a malignant giant cell tumor of soft parts.

An unusual case of a metastasizing dermatofibrosarcoma protuberans with fibrosarcomatous change (DFSP-FS) is reported. The tumor metastasized to unusual sites, including soft tissues of the cheek, forearm, and retroperitoneum 5 years after surgical resection without local recurrence. In addition, the patient had a second, histologically distinct fibrohistiocytic tumor: a malignant giant cell tumor of soft parts. The occurrence of these two tumor types in the same patient highlights the relationship of DFSP to the family of fibrohistiocytic tumors. The late onset of metastases in the absence of local relapse, although a rare event, reflects the potential difficulty in predicting its biologic behavior. DFSP-FS may be a more aggressive tumor than ordinary DFSP.

Immunohistochemical comparison of cutaneous lymphadenoma, trichoblastoma, and basal cell carcinoma: support for classification of lymphadenoma as a variant of trichoblastoma.

Cutaneous lymphadenoma is an uncommon basaloid epithelial tumor of uncertain histogenesis, most recently classified as a variant of trichoblastoma. Because characteristic immunohistochemical findings have been reported in trichoblastomas, we evaluated the staining patterns of five cutaneous lymphadenomas and compared the results to those of ten trichoblastomas and ten nodular basal cell carcinomas (BCCs), using antibodies to cytokeratin 20 (CK20), bcl-2, and CD34. In addition, because lymphadenomas contain intraepithelial S100-positive putative Langerhans cells, we compared staining of all tumor groups for S100 protein and CD1a. We also attempted to corroborate recent reports of CD30-positive activated lymphocytes in lymphadenomas. We identified CK20-positive Merkel cells in 3/5 lymphadenomas, 7/10 trichoblastomas, and none of the BCCs. Staining for bcl-2 accentuated the peripheral epithelial layer in all lymphadenomas and in 3/10 trichoblastomas, while the remaining trichoblastomas and all BCCs stained diffusely. There was stromal staining with CD34 in two lymphadenoma, 4 trichoblastomas, and 3 BCCs. All lymphadenomas featured numerous intraepithelial S100-positive cells which were also positive for CD1a in three cases tested. In addition, 8/10 trichoblastomas and 2/10 BCCs contained modest numbers of cells labelling for S100 and CD1a. Two of three lymphadenomas contained rare single cells resembling histiocytes faintly positive for CD30, and similar cells labelled for CD68. We conclude that the similar staining patterns of lymphadenomas and trichoblastomas support the classification of lymphadenoma as a variant of trichoblastoma. Staining with CD34 does not reliably distinguish between these tumors and BCCs. Lymphadenomas, trichoblastomas, and BCCs may all contain Langerhans' cells. The relationship between these cells and the striking lymphoid infiltrates seen in lymphadenomas is not clear. In our cases, the CD30-positive cells in lymphadenomas appear to represent histiocytes rather than activated lymphocytes.

Repair of a multiply recurrent giant cell reparative granuloma of the hand with wide resection and fibular grafting.

A patient with multiply recurrent giant cell reparative granuloma of the third metacarpal is reported. Three prior excisions failed to prevent recurrence. A wide resection and replacement with a nonvascularized fibular bone graft resulted in elimination of the tumor at the 7-year follow-up visit.

Fulminant herpes colitis in a patient with Crohn's disease.

Herpes simplex virus (HSV) is a well-recognized cause of gastrointestinal infection, most commonly in patients with underlying immunodeficiency. The esophagus, perianum, and rectum are the most common sites of involvement; however, extensive colitis is rare. We describe a woman with Crohn's disease who developed pathologically proven HSV colitis. We review the literature and present the possible implications of the diagnosis.

Colonic mucosal abnormalities associated with oral sodium phosphate solution.

BACKGROUND: Oral sodium phosphate (NaP) is increasingly used as a colonic cleansing agent for colonoscopy. It has been shown to be efficacious, well-tolerated, and safe. Mucosal abnormalities associated with NaP have recently been described. We carried out this controlled study to assess whether bowel cleansing preparations commonly used in colonoscopy are associated with colonic mucosal changes that may mimic inflammatory bowel disease (IBD). METHOD: All patients undergoing colonoscopy from January 1994 to June 1994 were considered for the study. Patients with history or symptoms suggestive of IBD were excluded. Patients were randomized to receive polyethylene glycol-based lavage (PEG-ELS) or NaP solution as their bowel cleansing preparation. Two gastroenterologists performing the colonoscopies were blinded to the type of preparation. Any mucosal abnormalities were noted and photographic documentation and biopsy specimens were taken. RESULTS: Ninety-seven patients were studied, 44 receiving PEG-ELS and 53 receiving NaP. Both groups were similar with regard to sex, age, and indication for colonoscopy. Fourteen patients were found to have nonspecific aphthoid-like erosions similar in appearance to Crohn's disease. These lesions, however, were not friable and biopsy results were not compatible with IBD. This mucosal abnormality was found in 13 patients who received NaP (24.5%) and only 1 patient who received PEG-ELS (2.3%). CONCLUSION: Nonspecific aphthoid-like mucosal lesions occur frequently in patients who received NaP for colonoscopy preparation. These lesions are endoscopically similar to those seen in Crohn's disease. Because of the potential for misinterpretation of these lesions, we do not recommend the use of NaP as a colonic cleansing preparation for patients with chronic diarrhea or in whom the diagnosis of IBD is suspected.

Renal failure and severe hypokalemia associated with acute myelomonocytic leukemia.

The leukemias have long been associated, albeit rarely, with the development of renal failure and several metabolic perturbations. While renal insufficiency may result from a variety of mechanisms in the setting of leukemia, severe leukostasis with microvascular insufficiency and renal parenchymal infiltration by blast cells are rare and infrequently described etiologies. In addition, hypokalemia can occur from lysozyme-induced renal tubular injury with inappropriate kaliuresis. We present a case of acute myelomonocytic leukemia that was complicated by renal failure and severe hypokalemia and discuss the probable mechanisms.

Cutaneous cryptococcosis in a patient with cutaneous T cell lymphoma receiving therapy with photopheresis and methotrexate.

Photopheresis is being used with increasing frequency as therapy for patients with neoplastic and dermatologic diseases and is being evaluated as therapy for patients with AIDS. We describe a patient with advanced cutaneous T cell lymphoma who developed pulmonary and cutaneous cryptococcosis after receiving therapy with photopheresis and biweekly methotrexate. We consider the potential roles of cutaneous T cell lymphoma, methotrexate, and photopheresis as predisposing factors in the development of serious cryptococcal infections.

The use of carbon marking after stereotactic large-core-needle breast biopsy.

PURPOSE: To investigate the use of activated charcoal to mark the biopsy site and needle track after large-core-needle breast biopsy. MATERIALS AND METHODS: Three hundred seventy-six consecutive patients (with 383 lesions) were referred for stereotactic breast biopsy. Two hundred forty-seven lesions were carbon marked when the need for surgery was likely. Patients who underwent marking were followed up for the results of surgery or mammography performed at our institution. Specimen sizes obtained by using the carbon mark were compared with sizes of consecutive biopsy specimens obtained after hook-wire localization. RESULTS: Carbon marking was well tolerated in all cases. All 132 surgeries performed at the authors' institution were successful in removing the marked target. Specimen sizes compared favorably with sizes of comparison hook-wire localization specimens. All 68 lesions followed mammographically revealed no changes that were attributable to the use of carbon. Two minor complications were observed. Two small cancers were completely removed at needle biopsy. CONCLUSION: Carbon marking is safe and effective for marking the biopsy site and needle track created by stereotactic large-core-needle biopsy of the breast. Marking eliminates the need for postprocedural needle localization. It remains effective when small lesions have been completely removed. This technique should be considered in properly selected cases by those performing large-core-needle biopsy of the breast.