Isolated intraosseous extra-gnathic orbital myxoma: a clinicopathologic case report.

A 19-year-old female student presented with painless, progressive proptosis in the right eye for the past six months causing significant cosmetic blemish and double vision. Clinical evaluation revealed normal visual acuity, normal pupillary reflex, severe proptosis, and dystopia. Orbital imaging revealed a heterogeneous mass in superior orbit displacing the globe inferiorly and causing extensive bony erosion of frontal bone superiorly and invading the frontal sinus. Excision biopsy was performed with en-bloc removal of the tumor. Surgical delivery of a slimy mass with infiltrated bony tissue led to a presumptive diagnosis of myxoid liposarcoma. Careful histopathologic analysis coupled with immunohistochemical studies were performed which established the rare diagnosis of low-grade myxoid spindle cell neoplasm with features suggestive of intraosseous myxoma. On follow-up at 15 months, our patient revealed no signs of recurrence. Isolated intraosseous orbital myxoma is a rare entity; only two such cases have been reported in the literature.

Improving Bethesda Reporting in Thyroid Cytology: A Team Effort Goes a Long Way and Still Miles to Go .

CONTEXT: Fine-needle aspiration cytology is the first step in evaluation of thyroid nodules. Although the Bethesda classification for reporting thyroid cytology has been purported that this uniformity in reporting cytology thereby facilitating clinical decision-making, there are also studies indicating that the reporting percentage and the rates of malignancy in each category vary considerably from center to center making the clinical decision more difficult. AIM AND MATERIALS AND METHODS: We looked at our retrospective cytology and histopathology data of thyroid nodules operated between 2012 and 2014 and then prospectively collected data during 2015-2016. In the prospective arm, for every thyroid nodule that was sampled, there was a discussion between the endocrinologist and the cytopathologist on the risk of thyroid cancer (based on the patient's history, examination findings, sonographic pattern, and the cytological appearance). RESULTS: We noted that there was considerable improvement in reporting standards with the rates of nondiagnostic cytology dropping from 11% to 5%, an increased reporting of Bethesda Category 2 and 6 which are the definitive strata of benign and malignant nodules (38% to 41% in Category 2 and 7% to 11% in Category 6) with a high specificity (100%). There was a decline in numbers of Category 4 and 5 (13% to 9% in Category 4 and 12% to 3% in Category 5). The reporting prevalence of Category 3 increased from 19% to 27%. CONCLUSIONS: We conclude that a team approach between the clinician who performs the ultrasound and the reporting cytopathologist improves Bethesda reporting, its predictive value, and thus potentially avoiding unnecessary thyroidectomies in benign thyroid nodules and hemithyroidectomies in thyroid cancers.

Plasmacytoid dendritic cells in lymph nodes of patients with human immunodeficiency virus.

Circulating plasmacytoid dendritic cells (PDC) decrease in human immunodeficiency virus (HIV) infection, either from loss or redistribution to lymph nodes (LN). Limited animal and human studies variably showed increased or decreased nodal PDC. CD123 immunostaining was performed on 28 archived LN biopsies (20 reactive) from 25 HIV patients. PDC clustering was graded (1: none; 2: rare small; 3: medium-sized, loose; and 4: large tight clusters) and correlated with HIV-lymphadenitis stage, blood CD4 counts, time since HIV diagnosis, and treatment duration. Increased PDC clustering was seen with decreasing CD4 counts (P = 0.001), shorter treatment duration (P = 0.0268), and advancing HIV-lymphadenitis stage (P = 0.06). No correlation with time since HIV diagnosis was noted. To our knowledge, this is the first human study assessing relationship of nodal PDC in HIV to CD4 counts, treatment duration, and lymphadenitis pattern. Our findings suggest that PDC redistribute to LN with advancing immunodeficiency and stage of HIV infection.

Clear cell sarcoma of the kidney--a study of seven cases over a period of three years.

UNLABELLED: Clear cell sarcoma of the kidney (CCSK) can display diverse morphological patterns and mimic various other pediatric renal tumors. An accurate diagnosis of this tumour is important considering the therapeutic and prognostic implications. AIM: The present study was undertaken to describe the various histological patterns of CCSK. The histology of 7 cases and the available case files of CCSK accrued over a period of3 years were reviewed. Immunohistochemical (IHC) stains were performed in 3 cases. The histological patterns observed in this study were classical (observed in 4 cases), epithelioid trabecular, myxoid, palisading and hyaline sclerosis types. IHC revealed reactivity to vimentin and non-reactivity to cytokeratin, desmin, smooth muscle actin, neuron specific enolase (NSE) and S-100 protein. Since CCSK is essentially a histological diagnosis, the importance of an accurate diagnosis of CCSK by a pathologist cannot be overemphasized. This study describes the various histological patterns that can be observed in CCSK.

Kimura disease with proliferative squamous metaplasia: an unusual finding and a potential diagnostic pitfall.

Kimura disease is a rare form of chronic inflammatory disorder involving subcutaneous tissue, predominantly in the head and neck region and frequently associated with regional lymphadenopathy and/or salivary gland involvement. We present a case of Kimura disease in a 28-year-old male which showed florid squamous metaplasia in the salivary gland ducts and salivary duct inclusions in the intraparotid nodes besides the usual features of Kimura disease. The squamous metaplasia was extensive enough to pose a diagnostic dilemma. We describe this case to highlight the rare histological finding of florid squamous metaplasia in Kimura disease and its diagnostic implications.

Cytological evaluation of head and neck tumors in children--a pattern analysis.

A total of 135 pediatric head and neck tumors diagnosed in our institute were reviewed with a view to elucidate the overall cytological patterns and analyze the important cytological features. Ninety-four tumors (69.6%) were aspirated for a primary diagnosis, and in 41 (30.4%) cases, fine-needle aspiration cytology was performed to document relapse, recurrence or a metastasis. Among the 94 tumors aspirated for a primary diagnosis, 66 cases (70.2%) were accurately diagnosed, in 22 cases (23.4%) a broad working diagnosis was offered, and 6 cases (6.4%) were misdiagnosed. The accuracy rate was higher (79.3%) when relapse-recurrent and metastatic tumors were included. The smears were broadly divided into six patterns, viz. round cell, epithelial, anaplastic, giant cell, mixed inflammatory, and spindle cell patterns. The round cell pattern was the most frequent one encountered in this group. The cytological features that stood the test of variability were lymphoglandular bodies and a noncohesive cell population in hematolymphoid malignancies, pale chromatin and cytoplasmic vacuoles in primitive neuroectodermal tumor/Ewing's sarcoma (PNET/ES), neuropil and rosettes in neuroblastoma, and plasmacytoid rhabdomyoblasts in rhabdomyosarcoma. A fairly good accuracy was seen in the diagnosis of metastatic undifferentiated carcinoma and anaplastic lymphoma, but the giant-cell and spindle-cell tumors continued to pose a problem in diagnosis. Ancillary techniques such as immunocytochemistry and electron microscopy applied in limited cases helped evaluate Langerhans cell histiocytosis, alveolar rhabdomyosarcoma, and the PNET/ES family of tumors.

Renal angiomyolipomas--a study of 18 cases.

Angiomyolipoma is a distinctive neoplasm composed of an intimate admixture of three components, viz. mature adipocytes, smooth muscle cells and blood vessels. This study was undertaken to better define the various morphological patterns of angiomyolipoma and their immunohistochemical profile. The paraffin blocks and slides of 18 cases of renal angiomyolipoma, accessioned over a period of 8 years from Tata Memorial Hospital, were reviewed. There were 2 men and 16 women in the age range of 17 to 68 years. Pre-operative fine needle aspiration cytology (FNAC) was performed in 6 cases, of which 5 were erroneously diagnosed as renal cell carcinomal sarcoma. Histologically, 14 cases revealed conventional histology. The remaining 4 cases were particularly misleading posing diagnostic problems due to variant patterns (leiomyomatous variant-3 cases and epithelioid variant- 1 case). Apart from the usual histology, necrosis, giant cells, and varying degrees of nuclear pleomorphism were present in three of the cases. Immunohistochemistry performed in 16 cases revealed immunoreactivity for HMB45 in all the 16 tumors (100%), estrogen receptor (ER) and progesterone receptor ((PR) in 2 cases (12.5%) and 6 cases (37.5%) respectively.

Desmoplastic round cell tumor of childhood: can cytology with immunocytochemistry serve as an alternative for tissue diagnosis?

There are limited reports on the cytology of desmoplastic small round cell tumors (DSRCT). Fine needle aspiration biopsy (FNAB) findings in seven aspirates from four cases of histologically and immunohistochemically confirmed cases were analyzed with the main intention of ascertaining if cytological diagnosis of DSRCT is possible. Also assessed were the immunocytochemistry(ICC) findings in these cases. The basic cytological impression was that of a cohesive small round cell tumor. Nuclei showed granular chromatin with grooves, nuclear molding and inconspicuous nucleoli. Stromal fragments were noted in all four cases. In two cases, awareness of cytological features in the appropriate clinical context led to a suggestion of the diagnosis of DSRCT on cytology itself. ICC on destained smears showed positivity for cytokeratin, epithelial membrane antigen (EMA), desmin and WT-1 in two cases. In conclusion, given the right clinical setting, a cytological diagnosis of DSRCT is plausible and in conjunction with ICC may help in documenting the polyphenotypic nature and thereby confirming the diagnosis.