Tag Archives: Conditions and Diseases

Langerhans Histiocytosis of the Skin

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  Langerhans cell histiocytosis is primarily a disease of childhood, although it clearly also occurs in adults, including the elderly. Classically, it is divided into three types: (i) Letterer-Siwe disease, which is prone to occur early in life and marked by multifocal skin and/or systemic involvement; (ii) Hand-Schüller-Christian disease, in which skin lesions can be accompanied by diabetes insipidus, exophthalmos, and bony defects; and (iii) unifocal disease, or eosinophilic granuloma, which affects older children and adults and in which skin involvement can accompany lesions of bones and other organs. Variant forms include an eruptive type limited to the skin that occurs in both infants and adults , a pulmonary form in young adults related to cigarette smoking , a form localized to the genitalia of elderly adults , and a rare multifocal type with marked cytologic atypia, termed Langerhans cell sarcoma . Another form presents at birth or shortly thereafter as single or multiple cutaneous papules and nodules that develop rapidly, ulcerate, and involute spontaneously; this variant is termed congenital self-healing reticulohistiocytosis . The appearance of lesions can range from crusted hemorrhagic papules in a seborrheic distribution (seen most often in Letterer-Siwe and Hand-Schüller-Christian types) to ulcerated plaques, papules, and nodules. Disease associations can include acute lymphoblastic leukemia, neonatal infection, lymphoma, and virus-associated hemophagocytic syndrome. Prognosis largely depends on the number of organ systems involved, with the poorest survivals being associated with multisystem disease.
Biopsy specimens show aggregates of cells with eosinophilic cytoplasm and nuclei displaying grooves and folds, sometimes producing a reniform appearance . These cells have a distinct tendency to infiltrate the overlying epidermis. In Langerhans cell sarcoma, cells are larger and possess nuclei with abnormal chromatin, prominent nucleoli, and frequent mitotic figures. Extravasated erythrocytes and inflammatory cells, including eosinophils, are frequently identified. The cells have the immunophenotype of Langerhans cells, expressing S-100 protein, CD1a, vimentin, and HLA-DR, and they also stain with peanut agglutinin. However, unlike normal intraepidermal Langerhans cells, the neoplastic cells express CD4 and placental alkaline phosphatase and show a different adhesion molecule expression profile. With electron microscopic analysis, Birbeck granules can be identified in a variable percentage of tumor cells. Morphologically, Langerhans cell histiocytosis can usually be distinguished from potential mimics such as xanthoma disseminatum and cutaneous T-cell lymphoma, and the immunohistochemical profile of its constituent cells is decisive. ( Sternberg’s 5th ed)

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ArterioVenous Malformation of the brain

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Arteriovenous malformations (AVMs) are  type of vascular malformation most relevant to the surgical pathologist. Because  their vessels tend to leak and induce gliosis.  AVMs can present with seizure disorder and also cause spontaneous hemorrhage. Best seen by trichrome or elastic stains, AVMs manifest artery-vein connections with associated arterialization of veins. Arterialization is thickening of a venous wall to the point where it resembles an artery, possibly due to fibrotic reaction to shunted arterial blood. Arteries show irregular duplication or loss of elastin . Thrombi are common. (Sternberg’s 5th ed)

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Renal Cell Carcinoma, Chromophobe Type “ChRCC”

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Hi there, few days ago i stumbled upon this relatively uncommon renal tumor and misdiagnosed it as Conventional renal cell carcinoma on smears. ” seams i got so much to learn ”

So lets review the Cytological Characteristics of this tumor :

  • polygonal cells.
  • well defined borders.
  • granular to light transparent cytoplasm, clear cells are rare.
  • accentuation of the granularity at the periphery of the cell.
  • central halo giving the cell koilocyte like appearance.
  • the nuclei are eccentric with anisonucleosis.
  • some of the nuclei are condensed with raisinoid appearance.

Beware of the Eosinophilic Variant of Chromophobe RCC as it very hard to differentiate from  Renal Oncocytoma which is a benign tumor.

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Ovarian SeroMucinous Cyst

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Ovarian cyst form 40 years old lady. it was 18 C.M in diameter, unilocular and filled with serous fluid.

on Aspirate you can see :

– Simple columnar epithelium ( the red spindly cells ” lets say pseudo-spindly always remember this is not a cross section “).

– the dispersed round cells in between on might think  they may represent the intercalated cells and then the diagnosis will be serous cyst But at high magnification you will see that they are full of foamy mucin with pushed nucleus typical of goblet cells.

– the foamy macrophages   ” last pic ” indicating the cystic nature of this aspirate

so we have 2 components the serous and mucinous and the proper diagnosis will be seromucinous cyst

please note that we can’t commet on the malignancy of such neoplasm unless we have H & E sections to asses invasion

Rt. Thyroid Nodule FNA

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55 years old female patient with history of breast carcinoma present’s with solitary thyroid nodule, spot diagnosis?

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