Small cell lung carcinoma with marked bronchial epithelial involvement.

Marked involvement of bronchial epithelium by malignant cells with a neuroendocrine immunophenotype was observed in a pulmonary lobectomy specimen containing combined small cell lung carcinoma (SCLC). Review of the medical literature reveals scant information on malignant neuroendocrine cells in bronchial epithelium accompanying SCLC and no documentation of an SCLC precursor. We discuss the possibility that the intraepithelial neoplastic lesion that we have described may be a primary lesion and possibly a precursor of SCLC and the alternative possibility that it represents invasion by underlying invasive SCLC. The need for further comprehensive study of the morphology and immunophenotype of bronchial mucosal abnormalities accompanying SCLC utilizing lung resection specimens is emphasized.

Female adnexal tumor of probable Wolffian origin (FATWO) with recurrence 3 years postsurgery.

This is the case report of a 38-year-old woman who presented with a mass of the right broad ligament that was diagnosed as a female adnexal tumor of probable Wollfian origin (FATWO). The patient was treated with a simple mass excision. Three years after the excision, the patient presented with uterine bleeding. A total abdominal hysterectomy was advised. Intraoperative histologic consultation showed a poorly differentiated tumor on the surface of the left ovary. After extensive immunohistochemistry analysis and after reviewing the histology slides from the primary tumor, the final diagnosis was concluded to be recurrent FATWO on the surface of the ovary. C-kit immunohistochemistry was found to be strongly positive. Polymerase chain reaction amplification of C-kit genes on exons 9, 11, 13, and 17 and of PDGFR gene on exons 12 and 18 showed no mutational changes. Owing to the limited options in treating recurrent disease and the lack of prognostic factors for recurrence or metastasis, the patient was started on 400 mg of imatinib mesylate therapy for 6 months. In addition, the patient is undergoing continuous follow-up by computed tomographic imaging every 6 months. As chemotherapy and radiation therapy for recurrent or metastatic FATWO are most often unsuccessful, a molecular targeted therapy, such as tyrosine kinase inhibitor, could be considered. However, collective data are needed from multiple centers to determine its effectiveness in these patients.

Facial herpes zoster complicated by varicella zoster virus (VZV) encephalitis: The diagnostic significance of atypical lymphocytes in cerebrospinal fluid (CSF).

BACKGROUND: In general, viral infections of the central nervous system (CNS) manifest as encephalitis and, less commonly, as meningoencephalitis or aseptic meningitis. Varicella zoster virus (VZV) is an uncommon cause of encephalitis. METHODS: Herpes zoster (shingles) is a cutaneous reactivation of previous chickenpox infection due to VZV. Herpes zoster may be dermatomal (ie, <3 dermatomes) or disseminated (ie, >3 dermatomes). Decreased cell-mediated immunity from stress, steroids, or immunosuppressive drugs often precede dermatomal/disseminated herpes zoster. With herpes zoster, the closer the dermatomal involvement is to the CNS (ie, head/neck shingles), the more likely a patient will have symptomatic CNS involvement (eg, encephalitis). Except for the association of the herpes zoster rash and the simultaneous/subsequent encephalitis, there are few clinical features that distinguish VZV encephalitis from that due to other viruses. The cerebrospinal fluid (CSF) profile of VZV encephalitis is usually clinically indistinguishable from that due to of other causes of viral encephalitis. In VZV meningoencephalitis or encephalitis, the CSF typically shows a modest lymphocytic pleocytosis with normal CSF glucose levels, variably elevated CSF protein levels, and normal CSF lactic acid levels. Atypical lymphocytes are rare in the CSF with VZV encephalitis. RESULTS: We present the case of a 75-year-old woman who developed VZV encephalitis after having herpes zoster on her forehead. Except for facial herpes zoster, there were no clinically distinguishing features to determine the cause of her encephalitis. Her CSF had 800 white blood cells/high power field with 26% lymphocytes (17% atypical lymphocytes). The patient's CSF glucose and CSF lactate dehydrogenase levels were normal, and her CSF protein was elevated. The CSF lactic acid was minimally elevated secondary to red blood cells in the CSF. Electroencephalogram showed general background slowing bilaterally, typical of viral encephalitis. The absence of unilateral focal frontotemporal/parietal lobe focus on electroencephalogram argued against the diagnosis of herpes simplex encephalitis. CSF atypical lymphocytes provided the key clue to the etiology of her encephalitis. CSF atypical lymphocytes are not uncommon in Epstein-Barr virus or cytomegalovirus encephalitis. Less commonly, atypical lymphocytes may be present in the CSF with enteroviruses, West Nile encephalitis, and Japanese encephalitis. VZV is a rare cause of atypical lymphocytes in the CSF but was the clue to the diagnosis before CSF polymerase chain reaction results for VZV were available. Her CSF polymerase chain reaction was negative for Mycobacterium tuberculosis, herpes simplex virus, human herpesvirus-6, cytomegalovirus, enteroviruses, and West Nile virus, but was positive for VZV. She made an uneventful recovery with acyclovir. CONCLUSION: CSF atypical lymphocytes, if present, are an important diagnostic clue in some causes of viral encephalitis. The most common cause of nonseasonal viral encephalitis is herpes simplex virus, which is not associated with CSF atypical lymphocytes. Patients with Epstein-Barr virus, cytomegalovirus, West Nile encephalitis, and enteroviruses usually have extra-CNS signs and symptoms which should suggest the cause of the patient's encephalitis. CSF atypical lymphocytes limit the differential diagnostic possibilities in patients with viral encephalitis and may be the key clue to the diagnosis, as in the case presented.

Swine influenza (H1N1) and acute appendicitis.

BACKGROUND: During the swine influenza (H1N1) pandemic that began in 2009, many hospitalized adults had gastrointestinal symptoms. The most common symptoms associated with swine influenza (H1N1) were nausea, vomiting, or diarrhea. In the experience of the authors, swine influenza was not complicated by abdominal pain. There are a wide variety of infectious and non-infectious disorders that may present with a pain in the right lower quadrant, mimicking appendicitis, ie, pseudoappendicitis. Influenza predisposes to some types of bacterial infection, eg, influenza pneumonia may be complicated by simultaneous Staphylococcus aureus community-acquired pneumonia or subsequent community-acquired pneumonia due to Haemophilus influenzae or Streptococcus pneumoniae. It remains unclear if there is direct involvement of the appendix, ie, pseudoappendicitis as occurs with measles or if influenza itself somehow predisposes to increased frequency/severity of bacterial appendicitis. German clinicians first noted an increased incidence of acute appendicitis in children/young adults with influenza. The American and British cases of influenza and acute appendicitis compared to age-matched controls with more severe and of delayed onset/complicated by appendicial perforation/abscess. These reports noted an increased incidence/severity of acute appendicitis during influenza. METHODS AND RESULTS: A 15-year-old girl presented to the hospital with an influenza-like illness and right lower quadrant abdominal pain. Acute appendicitis was diagnosed by a computed tomography scan and the patient underwent emergency appendectomy. Subsequently, it was noted that she did not have leukocytosis and, in fact, had borderline leukopenia. Her differential white blood cell count also revealed relative lymphopenia. Neither leukopenia nor relative lymphopenia are features of acute bacterial appendicitis. These two findings in the setting of an influenza-like illness indicate the underlying presence of influenza. Post-operatively, respiratory secretion samples were sent for swine influenza (H1N1) testing. Both her respiratory florescent antibody (FA viral panel) was positive for influenza A, as was her RT-PCR for swine influenza (H1N1). The authors believe that this is the first case of swine influenza (H1N1) and acute bacterial appendicitis. Direct involvement of the appendix by swine influenza (H1N1) virus could not be demonstrated. CONCLUSIONS: The authors conclude that during the swine influenza (H1N1) pandemic clinicians should be alert to the possibility of an increased incidence/severity of acute bacterial appendicitis in patients with swine influenza (H1N1) infection.

Scar endometriosis - a rare cause for a painful scar: A case report and review of the literature.

Endometriosis is described as the presence of functioning endometrial tissue outside the uterine cavity. Scar endometriosis is a rare disease, and is difficult to diagnose. The symptoms are nonspecific, typically involving abdominal wall pain at the incision site at the time of menstruation. It commonly follows obstetrical and gynecological surgeries. The diagnosis is frequently made only after excision of the diseased tissue. A case report of a patient with a troublesome scar after a caesarian section is presented. Surgical excision led to the diagnosis of scar endometriosis. The pathogenesis, diagnosis and treatment of this somewhat rare condition are discussed.

Fever of unknown origin (FUO) caused by Kikuchi's disease mimicking lymphoma.

Fever of unknown origin (FUO) refers to infectious, neoplastic, or rheumatic/inflammatory disorders that present with fevers of 101 degrees F or greater for 3 weeks and that remain undiagnosed after an intensive in-hospital or outpatient workup. The noninfectious causes of FUO in adults are most often lymphomas or rheumatic/inflammatory disorders. Among the rare causes of rheumatic/inflammatory FUOs is Kikuchi's disease. Kikuchi's disease (Kikuchi-Fujimoto disease) is also known as histiocytic necrotizing lymphadenitis, a benign, self-limited disorder usually in middle-aged women of Asian descent. Cervical adenopathy is typical and often accompanied by leukopenia. In middle-aged adults patients presenting with an FUO, the presence of otherwise unexplained cervical adenopathy should suggest the possibility of lymphoma or, rarely, Kikuchi's disease.

Oral paricalcitol in the treatment of patients with CKD and proteinuria: a randomized trial.

BACKGROUND: Vitamin D has key roles in regulating systems that could be important in the pathobiological state of proteinuria. Because of this, it could be helpful in treating patients with proteinuric renal diseases. The objective is to determine the effect of oral paricalcitol on protein excretion in patients with proteinuric chronic kidney disease. STUDY DESIGN: Double-blind randomized study. SETTING & PARTICIPANTS: 61 patients with estimated glomerular filtration rate of 15 to 90 mL/min/1.73 m(2) and protein excretion greater than 400 mg/24 h. INTERVENTION: Randomization to 6 months of treatment with paricalcitol, 1 mug/d, or placebo. OUTCOMES & MEASUREMENTS: The predefined primary end point was to compare change in mean spot urinary protein-creatinine ratio between the baseline measurement and the last study evaluation (6 months in study completers) between the 2 groups. Every 4 weeks, there was measurement of serum intact parathyroid hormone, serum calcium, serum phosphorus, serum creatinine, and urine spot protein and creatinine. RESULTS: At baseline, mean urinary protein-creatinine ratios were 2.6 and 2.8 g/g in the placebo and paricalcitol groups, respectively. At final evaluation, mean ratios were 2.7 and 2.3, respectively. Changes in protein excretion from baseline to last evaluation were +2.9% for controls and -17.6% for the paricalcitol group (P = 0.04). A 10% decrease in proteinuria occurred in controls (7 of 27; 25.9%) and the paricalcitol group (16 of 28; 57.1%; P = 0.03). LIMITATIONS: The relatively small sample size limits the extent to which results should be generalized. CONCLUSIONS: Paricalcitol resulted in a significant reduction in protein excretion in patients with proteinuric renal disease.

Fever of unknown origin (FUO) due to Rosai-Dorfman disease with mediastinal adenopathy mimicking lymphoma: diagnostic importance of elevated serum ferritin levels and polyclonal gammopathy.

Fever of unknown origin (FUO) characterizes febrile disorders that are accompanied by prolonged fevers of 101 degrees F or greater for 3 weeks or more that remain undiagnosed after comprehensive inpatient and outpatient diagnostic testing. At the present time, malignancies are the most common cause of FUOs. Among malignant FUOs, lymphomas are the most common. We present the case of a non-Asian young adult man who presented with FUO. He had no peripheral adenopathy or splenomegaly but was found to have anterior/superior mediastinal adenopathy and right paratracheal adenopathy. His diagnostic workup was negative for rheumatic/inflammatory and infectious diseases. Laboratory test results were unremarkable except for a highly elevated erythrocyte sedimentation rate and highly elevated serum ferritin level. Otherwise unexplained highly elevated serum ferritin levels in patients with FUOs suggest rheumatic and inflammatory disorders, for example, systemic lupus erythematosus flare or malignancy. The findings of mediastinal adenopathy combined with a highly elevated ESR and highly elevated serum ferritin levels indicate lymphoma as the most likely diagnosis. He also had polyclonal gammopathy on serum protein electrophoresis (SPEP). In a patient with FUO, negative blood cultures, and a heart murmur, polyclonal gammopathy on SPEP suggests atrial myxoma. Lymphomas are often associated with elevated alpha(1)/alpha(2) globulins on SPEP. Lymph node biopsy of the mediastinal nodes was negative for lymphoma but did not show characteristic emperiopolesis, pathognomonic of Rosai-Dorfman disease, a benign lymphoproliferative disorder. Rosai-Dorfman disease usually presents with massive bilateral cervical adenopathy but may present with lymph node involvement in other sites, as in this case. In patients with lymphadenopathy and a negative FUO workup, clinicians should consider the possibility of Rosai-Dorfman disease, particularly if accompanied by an otherwise unexplained highly elevated serum ferritin levels and polyclonal gammopathy on SPEP.

Radiology-Pathology Conference: choledochal cyst.

We report the radiologic and pathologic findings of a newborn with a choledochal cyst and review the etiology, diagnostic considerations, and management of this entity.

Quadrivalvular marantic endocarditis (ME) mimicking acute bacterial endocarditis (ABE).

Marantic endocarditis (ME) is defined by noninfectious valvular vegetations. The most common disorders associated with ME are malignancy with or without hypercoagulable state, intercardiac instrumentation, residual vegetations from previously treated infective endocarditis (IE), renal insufficiency, and burns. Another important cause of ME is systemic lupus erythematosus when accompanied by vegetations, that is, Libman-Sacks endocarditis. ME should be differentiated from IE because they may present with similar clinical features. Both ME and IE may present with fever and a heart murmur with or without embolic phenomenon. Leukocytosis and elevated erythrocyte sedimentation rate suggest the diagnosis of IE. The hallmark of IE is a cardiac vegetation and continuous high-grade bacteremia. After exclusion of the causes of culture negative endocarditis, the absence of bacteremia clearly differentiates ME from IE. We present a case of ME mimicking acute bacterial endocarditis (ABE). The differential diagnostic features of ME versus IE are discussed. To the best of our knowledge, this is the first reported case of quadrivalvular ME with massive vegetations on all cardiac valves, as well as the aorta, atria, and pulmonary artery.