Quiz 27: What is your diagnosis?

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Diagnosis: Quiz 27

Quiz 27

Answer:  Neurofibroma

Criteria for diagnosis clinically: A smooth-surfaced, somewhat acuminate pink papule with a slightly violaceous cast is a neurofibroma.

Differential diagnosis clinically: This could be any number of benign neoplasms, including a glomangioma or even an inflammatory process, such as a response to an assault by an arthropod. Biopsy is crucial to coming to a diagnosis with precision.

Criteria for diagnosis histopathologically: A broad, somewhat ill-defined nodule in the reticular dermis made up of delicate bundles of collagen, amphophyllic-staining, in company with numerous fibrocytes having a thin, oval nucleus and many Schwann cells displaying a somewhat squiggly nucleus, as well as scattered mast cells, along with dilated venules throughout the substance of the lesion, are findings of neurofibroma.

Differential diagnosis histopathologically: There is none.

Clinicopathologic correlation: The papule came into being because of the mass of collagen in the reticular dermis, the violaceous hue as a consequence of venules throughout the lesion having been dilated widely in vivo  and housing countless erythrocytes, and the surface is smooth because the stratum corneum is normal.

Options for therapy predicated on knowledge of histopathologic findings: A neurofibroma is benign and there is no need to remove it.

1) This reddish papule could be misinterpreted clinically as a vascular abnormality, ranging from a spider angioma to a hemangioma. In brief, a neurofibroma can present itself with a reddish cast instead of its more usual color like that of adjacent normal skin.

2) The neurofibroma pictured in this section of tissue is stereotypical of that benign nonepithelial neoplasm. The periphery, as is apparent here, is not circumscribed sharply. Not uncommonly, clefts form within the lesion itself, as is the case here. The numerous cells with a small thin nucleus are either fibrocytes or Schwann cells, the nucleus of the latter tending to have a subtle S-shape. Mast cells sprinkled throughout are findings expected in neurofibroma. In loci there are signs of neural differentiation in the form of structures that simulate tiny nerve fascicles.

3) On the basis of the findings histopathologic alone, no judgment can be made about whether or not this patient has neurofibromatosis. Only when a neurofibroma is plexiform histopathologically can it be determined that the patient who bears it has neurofibromatosis. In fact, however, this patient does have neurofibromatosis; in addition to many neurofibromas, there are prominent café au lait patches.

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