Kimura’s disease

A 12 year old child developed primary unilateral cervical mass. Routine investigations were inconclusive except cosinophibilia. Excision biopsy revealed the diagonisis as Kimura’s Disease. Retrospective investigations confirmed it. This disease should be suspected when a patient presents with tetrad of painless unilateral cervical lymphodenopathy. Eosinophilia, Hyperimmunoglobulimemia (††/gE) & Positive Candida Specific Antibodies. A flow chart has been drawn to treat this condition after review of literature.Key Words: Kimura’s, Candida specific Antibodies, Eosinophilia     

Paget disease of the breast—an easily overlooked disease?

BACKGROUND AND OBJECTIVES: Paget disease of the breast has long been recognized as a distinct clinical disease. The clinical manifestations and outcomes of Paget disease of the breast were reviewed to understand its earlier diagnostic clues and achieve an optimal treatment plan. Patients and Methods Thirty-one patients with Paget disease of the breast were included in this study. The postoperative outcomes and possible related prognostic factors were reviewed and analyzed. RESULTS: The 5-year overall survival was 69%. Nineteen patients (61%, Group A) did not have a palpable breast mass and 12 patients (39%, Group B) had a palpable breast mass. There was significant difference (P < 0.01) in the 5-year overall survival between Group A (94%) and Group B (19%). Group A patients had significantly higher incidences of underlying noninvasive breast carcinoma (73% vs. 8%, P < 0.01) and n0 lymph nodes status (84%vs. 50%, P = 0.043) than those of Group B. CONCLUSIONS: Paget disease of the breast without a palpable breast mass carries a more favorable prognosis. Patients with any nipple complaints deserve a detailed evaluation even in the absence of a palpable breast mass in order not to overlook a favorable disease.     

Early-stage hodgkin’s disease

Treatment of Hodgkin’s disease (HD) is strictly dependent on stage. Historically, early-stage HD included the limited stages I, II, and IIIA (according to the Cotswolds modification of the Ann Arbor classsification), whereas advanced HD included stage III with B symptoms and stage IV. It was then observed that early-stage HD with certain clinical risk factors had a significantly worse outcome. As a consequence, several studies defined these patients as suffering from early-stage unfavorable (or intermediate-stage) HD, demanding a more aggressive treatment. The treatment of early-stage HD is changing strikingly. Until recently, extended-field (EF) irradiation has been considered the standard treatment. However, because of the recognition of its high relapse rate and fatal long-term effects, EF radiotherapy is now being abandoned by most study groups. Instead, for favorable early-stage HD, mild chemotherapy for control of occult disease is combined with involved-field (IF) irradiation. In early-stage unfavorable (intermediate) HD, four cycles of chemotherapy plus IF irradiation is accepted as standard treatment.     

Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?

Idiopathic multicentric Castleman disease (iMCD) is a systemic disorder characterized by systemic inflammation and organ dysfunction associated with an increase in pro-inflammatory cytokines. Some patients with iMCD are positive for autoantibodies, although their significance and relationship with specific associated autoimmune diseases are unclear. This study retrospectively analyzed the clinicopathological features of iMCD patients focusing on autoantibodies. Among 63 iMCD patients in our database, 19 were positive for at least one autoantibody. Among the 19, we identified five with plasma cell type (PC)-iMCD lymph node histopathology and positive anti-phospholipid antibodies. These patients were likely to have thrombocytopenia, anasarca, fever, reticulin fibrosis or renal insufficiency, organomegaly (TAFRO) symptoms, and thrombotic events. The present study suggests that patients with undiagnosed or atypical autoimmune diseases, including anti-phospholipid syndrome (APS), were treated for iMCD. APS may present with thrombocytopenia or even multi-organ failure, which overlap with clinical presentations of iMCD. Due to differences in the treatment regimen and follow-up, recognition of the undiagnosed autoimmune disease process in those suspected of iMCD is essential. Our study highlights the importance of complete exclusion of differential diagnoses in patients with iMCD in their diagnostic workup.     

Lymphocyte predominant Hodgkin’s disease

Lymphocyte predominant Hodgkin’s disease (LPHD) is a rare type of B-cell lymphoma with unique pathologic and clinical features that distinguish it from other types of Hodgkin’s disease. Patients with LPHD tend to be younger males who present with indolent and asymptomatic lymphadenopathy limited to peripheral lymph nodes. The immunophenotype of the malignant lymphocytic and/or histiocytic cells (CD20+, CD15-, CD30-) forms the basis of the pathologic distinction from the subtypes of classical Hodgkin’s disease. Despite an excellent response to aggressive upfront combined-modality treatment, patients with LPHD tend to relapse continuously over decades. The benign nature of these relapses and the incidence of late treatment-related toxicity have raised questions about the need for an aggressive upfront approach. Recent insights into the molecular pathogenesis of LPHD and the development of novel targeted therapies promise to improve future treatment.     

Treatment of castleman’s disease

Opinion statement Castleman’s disease (CD) was first described in 1954 and further defined in 1956 by Castleman. Since then much has been learned about the heterogeneity of this condition. Subsequently, three pathologic classifications have been developed (hyaline vascular [HV] variant, plasma cell [PC] variant, and mixed variant) and two clinical classifications (unicentric [unifocal or localized] and multicentric [multifocal or generalized]). The pathology found with the unicentric presentation is most commonly that of the HV variant. It responds well to surgical resection and is associated with a benign course. The multicentric presentation is rarely composed of lymph nodes with HV pathology, but rather with the plasma cell or mixed pathology. This presentation requires systemic therapy and prognosis is guarded. Associated systemic symptoms are common. There is an increased incidence of CD in patients with HIV. The human herpes virus-8 is associated with nearly all of the HIV-associated CD cases and nearly 50% of non-HIV cases. Interleukin (IL)-6 has also been shown to play a significant role in the pathogenesis of the disease. Paraneoplastic and autoimmune entities are not uncommon in the disorder. Variable benefit has been achieved with single agent chemotherapy, combination chemotherapy, interferon (IFN)-α, rituximab, antiIL-6 receptor antibodies, and thalidomide. Patients with CD are at increased risk for developing frank malignant lymphoma.     

Gallbladder cancer, a vanishing disease?

Objective

Gallbladder cancer (GBC) is a rare gastrointestinal malignancy. A retrospective population-based study was conducted to evaluate trends in incidence, treatment, and outcome of GBC in the latter three decades in the south of the Netherlands.

Methods

All patients diagnosed with GBC diagnosed in the Dutch Eindhoven Cancer Registry area between 1975 and 2008 were included (n?=?659). Trend analyses were conducted for treatment and survival.

Results

During this time period, standardized incidence in females and males plummeted from 4.5 to 0.7 and from 2.0 to 0.4 per 100,000 inhabitants, respectively. Resection rates decreased from 74.3 to 53.4?%. Chemotherapy and radiotherapy rates did not change and were used sparingly. Five-year survival remained stable (10?%) over time.

Conclusion

The age-standardized incidence of GBC declined drastically over the last three decades. An increasing number of early cholecystectomies for gallstones may play a role. Parallel to the decreasing incidence of stomach cancer, the effective treatment of Helicobacter pylori may also have resulted in a lowered incidence of GBC.     

Pancreatic cancer–Surgery for recurrent disease

Recurrence of pancreatic ductal adenocarcinoma (PDAC) after a resection with curative intent is inevitable in the majority of cases. Approximately three-quarters of patients ultimately die from metastatic, local, or combined tumor recurrence [1–3]. This may be due to the insuf     

Paget’s disease of the nipple

The widespread use of trastuzumab in the past decade has led to a significant and measureable improvement in the survival of patients with human epidermal growth factor receptor-2 (HER2) overexpressing breast cancer, and in many ways has redefined the natural history of this aggressive breast cancer subtype. Historically, survival in patients with HER2-positive disease was dictated by the systemic disease course, and what appears to be the central nervous system (CNS) tropism associated with HER2-amplified tumors was not clinically evident. With improved systemic control and prolonged survival, the incidence of brain metastases has increased, and CNS disease, often in the setting of well-controlled extracranial disease, is proving to be an increasingly important and clinically challenging cause of morbidity and mortality in patients with HER2-positive advanced breast cancer. This review summarizes the known clinical data for the systemic treatment of HER2-positive CNS metastases and includes information about ongoing clinical trials of novel therapies as well as emerging strategies for early detection and prevention.     

Epstein-Barr virus and hodgkin’s disease

Approximately 40% to 50% of cases of Hodgkin’s disease occurring in Western populations are associated with the Epstein-Barr virus (EBV). In these cases, EBV is found in the neoplastic elements, the Reed-Sternberg and Hodgkin’s cells. EBV is probably not present in all cases, but neither have any other viruses been found in the cases that are EBV-negative. EBV may play a role in the pathogenesis of Hodgkin’s disease by the activation of anti-apoptotic factors in a premalignant germinal center B-lymphocyte. Regardless of their role in etiology or pathogenesis, EBV-latent antigens may represent a target for possible immune therapy.     

Treatment of human immunodeficiency virus-associated hodgkin disease is there a clue regarding the cause of hodgkin disease?

Background. The occurrence of human immunodeficiency virus (HIV)-associated Hodgkin disease (HD) offers a unique opportunity to study the cause of HD and compare HIV–HD with the well-characterized HIV–non-Hodgkin lymphoma (NHL). Methods. Eight patients with HIV–HD and 17 with HIV–NHL were treated. Results. The complete remission (CR) rate in HIV–HD was 100% with mechlorethamine, vincristine, procarbazine, and prednisone or doxorubicin, bleomycin, vinblastine, and dacarbazine (median survival, > 38.0 months). HIV–NHL patients were treated with cyclophosphamide, doxorubicin, vincristine, and prednisone (CR, 80%; median survival, 13.0±1.3 months). Durable CR was achieved with one to six cycles of chemotherapy (median, 4). There were no late relapses. The difference between the survival rate associated with chemotherapy-treated HIV–HD and chemotherapy-treated HIV–NHL approached statistical significance (P = 0.06). Analysis indicated that all patients with HIV–HD (n = 8) may have acquired HIV through intravenous drug abuse (IVDA) compared with 1 of 17 patients with HIV–NHL (P = 0.0001). A combined analysis (metaanalysis) of 157 patients with chemotherapy-treated HIV–NHL and 51 with chemotherapy-treated HIV–HD confirmed the significantly better survival of those with HIV–HD (P < 0.0001). Conclusions. Standard combination chemotherapy, truncated as necessary, offers survival outcomes that are at least equivalent and, perhaps, superior to previously published experimental approaches for HIV–NHL and HIV–HD. HIV–HD has a significantly better prognosis than HIV–NHL and is associated with IVDA. These data suggest that the etiologic agents of HIV–HD and HIV–NHL may be transmissible, identifiable, and unique.     

Isolated sphenoid sinus disease — a retrospective analysis

Objective

To evaluate the etiology, symptoms, signs, imaging, surgical findings and outcomes of isolated sphenoid sinus disease (ISSD).

Design

Retrospective study.

Settings

Tertiary university based referral center.

Materials and methods

All 8 patients aged 17–63, managed surgically in the department of ENT and Head and Neck Surgery at St. John’s Medical College and Hospital, Bangalore from 2006 to 2008 for ISSD. Demographic data, presenting signs and symptoms endoscopic and imaging findings, surgical management, surgical pathology and clinical outcomes were investigated in the above patients.

Results

Of the 8 cases of ISSD, 5 were male; 3 were female, with an age range of 17–63 years. The most common presenting symptom was headache (7 patients [87.5%]), followed by nasal obstruction and recurrent URTI (5 cases [62.5%]). Imaging included CT and/or MRI studies in all cases. Sphenoid sinus pathology was varied and included 5 (62.5%) inflammatory cases, 1 (11.1%) cerebrospinal fluid fistula and 2 (22.2%) cases of sphenoid sinus neop;asms. Of the inflammatory cases 2 (40%) had isolated polyps in the sphenoid sinus [sphenochoanal polyps] and 3 (60%) had fungal sinusitis. Treatment was surgical, endoscopic transnasal sphenoidotomy under general anesthesia in all 5 patients with inflammatory ISSD Two patients with sphenoid sinus tumors underwent endoscopic biopsy.

Conclusion

ISSD is rare. A high index of suspicion is required for diagnosis, which should be an active process and not one of exclusion. Both diagnostic nasal endoscopy and CT imaging are essential for diagnosis. The direct approach to the sphenoid sinus, transnasal endoscopic sphenoidotomy without ethmoidectomy is safe and effective. With early and adequate surgery we were able to avoid the morbidity associated with ISSD.     

Is mantle cell lymphoma a sex-related disease?

Mantle cell lymphomas are characterized by a male predominance with a range between 55 and 65 years (sex ratio M/F of 6.5). When the sex ratio of patients having mantle cell lymphoma was compared to that of each of the subtypes of non-Hodgkin's lymphomas, it was significantly higher in all cases except Burkitt's and lymphoblastic T-cell lymphomas. These observations suggest a possible relation between the chromosome X and mantle cell lymphomas which has to be explored.     

Bladder cancer,a two phased disease?

The processes of intraepithelial migration, intraluminal seeding, and field cancerization as models for initiation, spread, and recurrences of urothelial cell carcinoma (UCC) are reviewed in light of recent molecular investigations. The accumulated molecular data on synchronous and metachronous tumors indicate that the majority of recurrent and multiple tumors are monoclonal. Molecular data has also shown the presence of chromosomal and genetic changes in precursor lesions as well as in normal urothelial cells. Genetic-histological mapping of cystectomized bladders has shown that overt tumors occur as local events in areas of genetically altered urothelium. A model is put forward in which the tumor process is initiated by genetically altered but histologically normal cells that produce fields of altered cells by intraepithelial displacement. By the accumulation of further genetic changes the fields of altered urothelium reaches a state of criticality, which locally may produce frank tumors.