Extranodal follicular dendritic cell sarcoma of the lung.

Follicular dendritic cell sarcoma (FDCS) is a rare, mesenchymal neoplasm that may be nodal or extranodal in location. Lung involvement is rare. It is a slow-growing, painless tumor with a frequent capacity to recur and metastasize. We present a case of extranodal FDCS of the lung with an unusual presentation. A 34-year-old man presented with the complaints of haemoptysis and chest pain. A large left perihilar mass with endobronchial component was found on radiological evaluation. On endobronchial biopsy and mediastinal tru-cut biopsy, differential diagnoses of an inflammatory myo-fibrohistiocytic lesion and leiomyosarcoma were provided on the basis of morphological features of bipolar spindled cells arranged in intersecting fascicles and storiform patterns and immunophenotyping. A pneumonectomy was performed for the mass on which further immunohistochemical evaluation with CD21, CD35, and D2-40 finally helped form a diagnosis of FDCS. The patient recovered well from the surgery and has been on follow-up ever since. Owing to the rarity of this condition and its non-specific clinical features, FDCS is often misdiagnosed in the absence of appropriate immunohistochemistry. An of awareness of its morphological features and immunophenotype is, thus, necessary to provide early treatment and follow-up in order to prevent its recurrence and metastasis.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: an under-recognized entity-a report of two cases.

Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare pulmonary disorder that is classified as a pre-invasive lesion. It can also be present in association with chronic lung diseases. We present two cases of DIPNECH, which were picked up incidentally only on histopathology. A 29-year-old male presented with a history of longstanding cough with expectoration. A computed tomography (CT) scan showed bronchiectasis in the left lung. Histopathological evaluation of the pneumonectomy specimen revealed nests of proliferating, non-infiltrating neuroendocrine cells. Our second patient was a 38-year-old female with chronic cough and expectoration with recent exacerbation. CT scan showed cystic bronchiectasis in the left lower lobe; hence, a lobectomy was performed. On histopathology, nests of non-infiltrating neuroendocrine cells were noted. A diagnosis of DIPNECH with bronchiectasis was offered to both patients, and they have been on follow-up ever since. DIPNECH is a diagnostic and therapeutic quandary requiring a high index of suspicion for its detection and effective surveillance.

Thoracic follicular dendritic cell sarcoma - an outlandish presentation of a rare tumour with review of literature.

Follicular dendritic cell sarcoma is a rare low grade malignant neoplasm that arises from follicular dendritic cells in lymphoid tissue germinal centres and accounts for 0.4% of all soft tissue sarcomas. It is extremely rare to have pulmonary follicular dendritic cell sarcoma with endobronchial extension and as an anterior mediastinal mass with mediastinal lymph node involvement. We present the case of a 34-year-old male non-smoker who had been experiencing chest pain for three months. A lobulated left peri-hilar mass with endobronchial spread into the left main bronchus and mediastinal lymphadenopathy was identified on a chest CT. The bronchoscope-guided cryobiopsy of the endobronchial mass was inconclusive. After a thorough multidisciplinary discussion, the patient underwent left sided pneumonectomy, mediastinal mass resection, and systematic lymph node dissection. Histologic examination using immunohistochemistry revealed follicular dendritic cell sarcoma.

Pulmonary Tumourlets: Case Report and Review of Literature.

We report a case of tumourlets of the lung associated with carcinoid and neuroendocrine cell hyperplasia, found incidentally in a 30-year-old woman, who underwent bullectomy for pneumothorax. These lesions are histologically similar to carcinoid, but differ in molecular pathogenesis about which little is known. Their nature and significance is debated. Here, we point out the importance of histological, clinical, and diagnostic aspects and follow-up to have evidence of eventual malignant evolution.

Mediastinal Teratoma with Coexisting Adenocarcinoma and Carcinoid Tumor (Somatic-Type Malignancy): A Case Report with a Review of the Literature.

Germ cell tumors constitute 10% to 15% of anterior mediastinal neoplasms. Of these, mature teratoma is the most common. Somatic malignant transformation in mature teratoma is a very rare phenomenon. In the anterior mediastinum, few cases of malignant transformation in the form of carcinoma, sarcoma, or neuroendocrine tumors have been described. We present the case of a mature mediastinal teratoma in a 24-year-old female, diagnosed on computed tomography, where both carcinoid tumor and adenocarcinoma were seen. To the best of our knowledge, this is the first report of such a case. Malignant transformation in a mature teratoma confers a significantly worse prognosis and is difficult to diagnose only on clinical and radiological evaluation. As these lesions are so rare, the treatment options for these lesions are also not clearly defined. Extensive sampling and careful microscopic examination are needed when teratomas are submitted for pathological evaluation.

Adenocarcinoma (somatic-type malignancy) in mature teratoma of anterior mediastinum.

Mature teratoma is a common anterior mediastinal tumour. However, occurrence of transformed malignant component within it is very rare. We report a case of a 32-year-old female presenting with dry cough and chest pain. Contrast-enhanced computed tomography (CT) showed a large thin-walled cystic lesion measuring 11.4 cm x 10.6 cm x 10.0 cm in the anterior mediastinum. Right postero-lateral thoracotomy was performed and the tumour was completely excised. Histopathological examination of the excised specimen was suggestive of mature teratoma with transformed malignant component, adenocarcinoma (somatic-type malignancy).

Primary synovial sarcoma of the mediastinum : a case report.

Synovial sarcomas commonly occur in the extremities of young adults. A primary occurrence in the mediastinum is very rare with only a few reported cases in the world literature. This paper is about a 42-year-old male who presented with chest pain and dyspnoea on exertion. Imaging showed an anterior mediastinal mass with adhesions to the lung. Pathological examination of the resected mass showed a biphasic neoplasm with a spindle cell component admixed with gland-like elements. The tumour showed positive staining with cytokeratin, epithelial membrane antigen, and Bcl-2 confirming the diagnosis of a biphasic synovial sarcoma. A wide range of neoplasms, both primary and metastatic, occur in the mediastinum, which pose considerable diagnostic difficulties. A synovial sarcoma should always be considered in the differential diagnosis, and immunohistochemistry is an important adjuvant tool in this situation. This paper highlights the importance of recognizing an unusual presentation of this aggressive neoplasm to aid appropriate clinical management.

Correlation of antinuclear antibody immunofluorescence patterns with immune profile using line immunoassay in the Indian scenario.

BACKGROUND: Immunity status, individual response to disease and types of antibodies produced are well known to vary from person to person, place to place and probably from population to population. A broad spectrum of specific auto antibodies that have so far been associated with specific rheumatic diseases, as noted in Western literature, has been well taken as a reference standard all over the world. There is neither research work nor any data correlating the auto antibodies and their antinuclear antibody (ANA) patterns with the immunoprofile in the Indian population to date. AIMS: To understand a definite association between ANA patterns and specific antibodies in the serum in the Indian study population and to document similarities / differences with the West. SETTINGS AND DESIGN: This prospective and retrospective double blind study was undertaken on the South Indian population referred for ANA testing by Indirect Immunofluorescence method and by immunoline methods. MATERIALS AND METHODS: Serum samples of patients from a random South Indian population who sought medical help for rheumatic disease were subjected for ANA testing by indirect immunofluorescence (IIF) method and line immunoassay during the study period of 27 months. Serum samples were processed in dilution of 1:100 using HEp - 2010 / liver biochip (Monkey) (EUROIMMUN AG). The serum samples which were further processed for line immunoassay were treated in 1:100 dilution on nylon strips coated with recombinant and purified antigens as discrete lines with plastic backing (EUROIMMUN AG) coated with antigens nRNP / Sm, Sm, SSA, Ro-52, SSB, Scl-70, PM-Scl, PCNA, Jo-1, CENP-B, dsDNA, nucleosomes, histones, ribosomal protein-P, anti-mitochondrial antibodies (AMA-M2) along with a control band. The analysis was done by comparing the intensity of the reaction with positive control line by image analysis. RESULTS: The antinuclear antibody indirect immunofluorescence (ANA - IIF) patterns obtained were projectable to visualize a certain spectrum of specific antibodies such as homogenous (45.5%) with dsDNA, nucleosomes, histones, SSA / Ro-52, RIB and RNP / Sm, speckled pattern (35.6%) with Sm, RNP, SSA/Ro-52, SSB, Sm and RIB; nucleolar pattern with Scl-70, Sm, RNP and centromere pattern with CENP-B. The methodology indicated that, cytoplasmic pattern noted in ANA also needs to be correlated with primate liver in a biochip, which should prompt further decision for a request for line immunoassay and it is preferable for two pathologists to report independently and sign out a consensus ANA report for better predictive value. CONCLUSIONS: As a definite correlation between the ANA patterns and the group of antibodies was detected by line immunoassay, one could predict presence of certain specific auto antibodies for a particular ANA pattern identified. This may restrict one from requesting for line immunoassay, which is expensive and economizes on the cost of laboratory investigations in a developing country like India. Thus, screening of sera by ANA-IIF method alone may suffice and probably reduce the expense of detailed immunological work-up with minimal loss in diagnostic accuracy. This study, the first of its kind in India, provides database and reference for the Indian subpopulation.

Impact of iron deficiency anaemia on T lymphocytes & their subsets in children.

BACKGROUND & OBJECTIVES: While there is evidence of an altered immune profile in iron deficiency, the precise immunoregulatory role of iron is not known. Information particular in children who are vulnerable to iron deficiency and infection, is lacking. We undertook this study with the aim of documenting the changes in T cell subsets in children in the age group of 1 to 5 yr with iron deficiency. METHODS: The levels of T lymphocytes, their CD4+ and CD8+ subsets and the CD4 : CD8 ratio were evaluated in 40 iron deficient and 30 healthy children. The impact of oral iron supplementation for three months on the same parameters was also noted in 30 children. RESULTS: Significantly lower levels of T lymphocytes as well as CD4+ cells was observed in the iron deficient children (P<0.01 and 0.002 respectively). The CD4 : CD8 ratio was also significantly lower in this group (P<0.05). Iron supplementation improved the CD4 counts significantly. INTERPRETATION & CONCLUSION: Our study demonstrated quantitatively altered T cell subsets in iron deficiency in children, and a relationship between the severity of haematological and immunological compromise. The clinical and epidemiological implications of this relationship have topical relevance since ID is the most common micronutrient deficiency worldwide.