The 10-year results after national introduction of pelvic lymph node staging in Danish intermediate-risk endometrial cancer patients not given postoperative radiotherapy.

The 10-year results after national introduction of pelvic lymph node staging in Danish intermediate-risk endometrial cancer patients not given postoperative radiotherapy. Gitte Ørtoft; Claus Høgdall; Estrid S Hansen; Margit Dueholm. OBJECTIVE: To prepare for the national introduction of sentinel node staging, we evaluated the consequences of the previous national decision to introduce lymph node staging in intermediate-risk endometrial cancer patients (grade 1/2 with > 50% or grade 3 with < 50% myometrial invasion) by determining the number of patients upstaged by lymphadenectomy and whether upstaging affected the survival and recurrence patterns of non-staged patients and patients with and without lymph node metastases. STUDY DESIGN: In a national cohort study, 2005-12, 1294 stage I-IV patients who should have been offered lymphadenectomy were progressively registered. The number of patients upstaged by lymphadenectomy, 10-year survivals were evaluated by Kaplan-Meier analysis and adjusted Cox regression. RESULTS: This study demonstrates that it takes time to introduce lymphadenectomy at a national level, as indicated by the increasing number of cases staged per year, from 12% in 2005 to 74% in 2012. Pelvic lymphadenectomy was performed in 43.8% (567/1294) and lymph node metastases were found in 13.6% (77/567). As 54 patients had further dissemination outside the uterine body, only 23 patients (6%) were upstaged from stage I to IIIC. Compared to lymph node-negative patients, the 77 patients with lymph node metastasis had significantly lower overall, (55% versus 68%), disease-specific (64% versus 86%), and progression-free survival (51% versus 77%), mainly due to non-local recurrences including a high number of paraaortic recurrences. In 873 final stage I intermediate-risk patients, 10-year survival and recurrence rates were not significantly lower in non-staged as compared to lymph node-negative patients (overall survival 62% versus 70%: disease-specific survival: 90% versus 90%, progression-free survival: 81% vs 83%), probably due to the low number of patients upstaged from stage I to stage IIIC. CONCLUSION: Lymph node metastases were present in 13.6% of patients with intermediate-risk who underwent pelvic lympadenectomy, and these patients had a lower 10-year survival than lymph node-negative patients. Because lymphadenectomy upstaged only 6% from stage I to stage IIIC, survival and recurrence rates were not significantly compromised in non-staged as compared to lymph node-negative intermediate-risk stage I patients. Sentinel node staging has now been implemented in Danish intermediate-risk endometrial cancer patients.

Lymph node abscess and cardiac involvement in a patient with nodular lepromatous leprosy (LL) with erythema nodosum leprosum (ENL): a rare occurrence.

With the world's focus on reducing the leprosy patient load to the extent of elimination, finding and reporting the rarer presentations of leprosy becomes important for prompt treatment. Also, these untreated patients may serve as a potential source of infection in community. We report a 35-year old man diagnosed to have lepromatous leprosy and erythema nodosum leprosum with inguinal lymph node abscess and suspected cardiac involvement that proved fatal. We stress the importance of detailed workup to look for associated systemic involvement for timely intervention and favourable outcome.

Expression of stabilin-1 in M2 macrophages in human granulomatous disease and melanocytic lesions.

Stabilin-1 is an endocytotic scavenger receptor, specifically expressed by non-continuous sinusoidal endothelial cells in the liver, spleen and lymph nodes and by M2 or alternatively activated macrophages in human malignancies. We analysed paraffin-embedded tissue of melanocytic lesions and granulomatous diseases for stabilin-1 expression, using the human/murine RS1 antibody. The specificity of the RS1 staining was confirmed in a knockout model, as only M2-like tumor-associated macrophages and vessels of a B16F10 melanoma in wild type mice stained positive; while staining of tumor-associated macrophages and vessels originating from stabilin-1 deficient mice remained negative for stabilin-1 specific antibody RS1. In human specimens, the RS1 antibody stained tumor-associated macrophages in all pathological stages of melanoma. In addition, five cases of juvenile xanthogranulomas and one case of necrobiotic xanthogranuloma were strongly stabilin-1 positive, while Th-1 cytokine dominated granulomatous diseases such as sarcoidosis and granulomatous leprosy were negative. Stabilin-1 positive vessels were found in all analysed non-Langerhans cell histiocytoses and melanocytic lesions. No stabilin-1 positive vessels were present in any other granulomatous diseases.

Anaplastic large cell lymphoma and lepromatous leprosy: a rare coexistence.

Lepromatous leprosy (LL) has been reported in the literature with Non Hodgkin Lymphoma and rarely with Hodgkin Lymphoma. However, an extensive search of the literature shows no case report describing anaplastic large cell lymphoma (ALCL) in association with LL. We report a case of a young male with LL who was found to have ALCL. This is an interesting case of coexistence of an endemic infectious disease and a rare lymphoma involving the same lymph node, with a brief review of the literature.

Type II reaction without erythema nodosum leprosum masquerading as lymphoma.

Lepromatous leprosy is a multisystem disease that can involve many organ systems, with lymph nodes a common extra-cutaneous site to be affected. Rarely, multibacillary leprosy can be confused with other diseases like lymphomas and connective tissue diseases. Herein we report a patient of lepromatous leprosy with Type II lepra reaction involving lymph nodes who presented with generalised lymphadenopathy, acquired ichthyosis and constitutional symptoms but no cutaneous lesions to suggest erythema nodosum leprosum, and who was initially misdiagnosed as a case of Hodgkin's lymphoma.

Lepromatous leprosy masquerading as acute sarcoidosis: a case report and literature review.

Leprosy is uncommon in North America. Because it has a prolonged incubation period and can masquerade with a variety of manifestations, many patients with leprosy experience a significant delay in diagnosis and treatment. Lepra reactions are of 2 types: reversal (type 1) and erythema nodosum leprosum (ENL) (type 2). Type 1 or reversal reactions represent an increase in cell-mediated immunity, whereas type 2 or ENL is caused by antigen-antibody complex formation and deposition after antigen release from dying lepra bacilli. This article describes the diagnostic challenges presented by a Minnesota patient eventually found to have lepromatous leprosy. That challenge was compounded by the fact that the clinical scenario closely mimicked connective tissue/immune complex disease and by the fact that the patient presented in a location where the incidence and prevalence of leprosy is extremely low.

Antigen-specific cellular and humoral responses are induced by intradermal Mycobacterium leprae infection of the mouse ear.

Leprosy is caused by infection with Mycobacterium leprae. The immune response of leprosy patients can be highly diverse, ranging from strong cellular responses accompanied by an apparent deficit of M. leprae-specific antibodies to strong humoral responses with a deficit of cell-mediated responses. Leprosy takes many years to manifest, and this has precluded analyses of disease and immune response development in infected humans. In an attempt to better define development of the immune response during leprosy we have developed an M. leprae ear infection model. Intradermal inoculation of M. leprae into the ear supported not only infection but also the development of a chronic inflammatory response. The inflammatory response was localized, comprising a T-cell infiltration into the ear and congestion of cells in the draining lymph nodes. The development of local chronic inflammation was prevented by rifampin treatment. Importantly, and in contrast to subcutaneous M. leprae footpad infection, systemic M. leprae-specific gamma interferon and antibody responses were detected following intradermal ear infection. These results indicate the utility of intradermal ear infection for both induction and understanding of the immune response during M. leprae infection and the identification or testing of new leprosy treatments.

The "sign of groove", a new cutaneous sign of internal malignancy.

A 36-year-old young male with multiple heterosexual contacts presented with bilateral inguinal bubo and the classical "sign of groove". A diagnosis of lymphogranuloma venereum (LGV) was made and a three-week course of doxycycline was given. Lack of response prompted us to investigate further. A biopsy of the bubo was consistent with non-Hodgkin's lymphoma (NHL). Immunohistochemistry of the lymph node done at the Regional Cancer Center (RCC), Trivandrum, confirmed the diagnosis as NHL of diffuse large B-cell type. The second patient, a 32-year-old male with two unprotected heterosexual contacts presented with a left-sided inguinal bubo of six weeks duration. An empirical course of doxycycline was given even though investigations did not reveal any STI. Lack of response prompted us to do a lymph node biopsy, which was consistent with NHL, which later with immunohistochemistry was confirmed as NHL, diffuse large cell type. We are reporting here that the "sign of groove" is not specific for LGV as thought earlier, but can occur in NHL also.

Cutaneous sarcoidosis: clinical profile of 23 Indian patients.

BACKGROUND: Sarcoidosis is a multisystem disease of undetermined etiology. Indian studies on cutaneous sarcoidosis are not many and mainly comprise case reports. AIMS: This retrospective study was carried out to assess the clinical profile of sarcoidosis patients presenting with cutaneous lesions. METHODS: All histopathologically proven cases of cutaneous sarcoidosis seen consecutively between 1999 and 2004 were studied. Their age, sex, presenting features, evolution of disease and laboratory parameters were analyzed. RESULTS: A total of 23 patients (F:M 15:8) between 31 to 78 years (mean 44.3 years) of age had the mean duration of skin lesions of 1.4 years. Six patients had one to four lesions; two patients each had scar sarcoidosis and angiolupoid and one patient each had recurrent erythema nodosum, leg lymphedema and subcutaneous sarcoidosis. Others showed combination of papules, nodules, plaques and psoriasiform lesions. Peripheral lymph nodes were involved in two patients. Among 10 patients of pulmonary involvement, three had become symptomatic four months to four years after the cutaneous lesions. Routine laboratory investigations including serum calcium estimation were normal in all cases. Serum angiotensin-converting enzyme levels were raised in 3 out of 6 patients. Asymptomatic lytic lesions of digital bones were detected in hand X-ray of one patient. CONCLUSION: Skin lesions of sarcoidosis are like the tip of an iceberg indicating more changes in other organs. The symptomatology and abnormal laboratory results do not necessarily correlate with the severity of cutaneous involvement in general.

Comparative cytomorphology of skin, lymph node, liver and bone marrow in patients with lepromatous leprosy.

OBJECTIVE: The aim of this study was to compare the cytological changes in skin, lymph nodes, liver and bone marrow in patients with lepromatous leprosy. METHODS: Skin lesion, lymph node, liver and bone marrow aspirates were analysed. May-Grunwald-Giemsa (MGG) and Ziehl-Neelsen (Z-N) stains were employed. Comparative cytomorphology was studied. RESULTS: Twenty patients with lepromatous leprosy were studied. Lepra cells (LC) predominated in the skin aspirates of 12 patients with lepromatous leprosy (LL), lymphocytes accompanied LC in eight patients with borderline-lepromatous (BL) leprosy. Three patients of LL leprosy and two of BL leprosy in type 2 reaction additionally had numerous neutrophils. Two patterns of lymph node aspirates were seen: partial replacement with few LC in a reactive lymphoid background (10), complete replacement with either only LC or LC in a background of degenerating neutrophils (10), the latter a feature of type 2 reaction. Liver aspiration was performed in seven patients and of bone marrow in eight patients. Occasional LC were present in five liver-aspirated patients, steatosis and Kupffer cell hyperplasia in four patients, and myelopoiesis in two patients. Bone marrow smears invariably had occasional LC and a relative increase in mature plasma cells; sea-blue histiocytes were seen in six patients. CONCLUSION: Lepra cells predominated in skin and lymph node aspirates with complete replacement. In comparison, liver, bone marrow and lymph node aspirates with partial replacement were dominated by a preponderance of cells native to these organs with only few or occasional LC.

Lepromatous lymphadenitis masquerading as lymphoma.

A 70-year-old male presented with multiple lymphadenopathy and a strong clinical suspicion of non-Hodgkin's lymphoma. Cervical and axillary nodes were excised and were sent for histopathological evaluation, which revealed aggregates of lepra cells loaded with lepra bacilli. Clinicians practising in leprosy endemic areas should keep lepromatous lymphadenitis in mind while investigating patients with lymphadenopathy.

Kikuchi's disease associated with systemic lupus erythematosus.

A 25-year-old multiparous female presented with fever, joint pains, facial rash and lymphadenopathy of three months' duration. Lymph node biopsy revealed a diagnosis of Kikuchi's disease. She fulfilled seven out of the 11 ARA criteria for SLE. The association of Kikuchi's disease and SLE is rare.

Rosai-Dorfman syndrome.

Rosai-Dorfman syndrome is characterized by sinus histiocytosis with massive lymphadenopathy. A 25-year-old woman presented with multiple erythematous and yellowish papules on the forehead, cheeks, chin and thigh. She had massive generalized, firm, non-tender and non-matted lymphadenopathy and mild hepatomegaly. Her hemogram was normal. A skin biopsy showed collections of histiocytes with emperipolesis and plenty of plasma cells. A lymph node biopsy showed partial loss of architecture, dilated sinuses filled with histiocytes, which showed lymphocytophagocytosis, anisonucleosis and a clear cytoplasm. She was referred to an oncologist for further management.