Experience...Dedication...Diagnostic Accuracy

Understanding the Jargon: What is a Verocay Body?

Within the lexicon of dermatopathology, as within any specialty, is terminology that may be unfamiliar to the clinician. One such term is the Verocay body. The usage of this wording becomes even more enigmatic when one considers both the Verocay Body and Verocay body-like structures as it relates to various pathologic entities.

In the August 2011 edition of The American Journal of Dermatopathology, Jag Bhawan and colleagues discuss the various cutaneous neoplasms that may present with Verocay body-like structures (1). A number of heterogeneous tumors have been known to demonstrate this feature, and include epithelial, adnexal, fibrohistiocytic, mesenchymal and melanocytic lesions.

The peculiar arrangement of nuclei in transverse rows in a nerve sheath tumor was first described by Verocay in 1910 (2). This later came to be known eponymously as the Verocay body, referring to the stacked arrangement of elon-gated, palisading nuclei alternating with anuclear zones composed of cytoplasmic processes (Refer to Case 14, page 4). This observation has stood the test of time as a valuable clue for the diagnosis of nerve sheath tumors.

An unusual skin appendageal tumor with similar alternating areas of epithelial cords and stroma,coined a “rippled pattern”, was described by Hashimoto, et al. Since then, a variety of histogenetically diverse neoplasms have been described to contain this “Verocay body-like” pattern. Non-neural tumors that most frequently exhibit this pattern include trichoblastoma, sebaceoma, basal cell carcinoma, dermatofi-broma, leimyoma and very rarely, melanocytic nevi.

The rippled Verocay body-like pattern is often seen in cases of sebaceoma. These tumors show a male predominance and an affinity to arise on the scalp (as opposed to the face), when compared with sebaceomas that lacked this pattern. Within the spectrum of adnexal tumors with a rippled pattern, it is important to make the distinction between sebaceoma and trichoblastoma in view of the association of the former with Muir-Torre syndrome.

Considering the close relationship between melanocytes and Schwann cells, it is surprising that only very rarely do nevi (less than 1 of many thousands encountered in dermatopathology services) show the distinctive Verocay body-like structures that are described herein (Refer to Case 9, page 3). This finding may also be seen rarely in cases of malignant mela-noma, where the schwannian pattern reflects the protean na-ture of these lesions.

References

(1) A Biswas, M.D, N Setia, M.D., J Bhawan, M.D. Cutaneous neoplasms with prominent Verocay body-like structures: the so-called “rippled pattern”. Am J Dermatopath; Vol 33:6, 2011; pp 539-48.

(2) Verocay J. Zur Kenntnis der “neurofibrome” [in German]. Beitr Pathol Anat.1910; 48:1-69.

Reena Sachdev, M.D., New Dermatopathologist Joins Clin-Path Team

The Dermatopathology Department of ClinPath Associates is pleased to introduce Reena Sachdev, M.D. as a new member of their team.  Board certified in dermatology and dermatopathology, Dr. Sachdev completed her fellowship training in dermatopathology at Stanford University in Palo Alto, California.  She completed a residency program in Dermatology at Albert Einstein College of Medicine in Bronx, New York.  Dr. Sachdev completed medical school at Michigan State University in East Lansing, Michigan.  Please join us in welcoming Dr. Sachdev to Phoenix.

Sebaceous Neoplasms: A Potential Sign for Internal Malignancies

Skin findings can often be the first sign of inheritable genetic syndromes, highlighting the pivotal role of dermatologists in diagnosing such conditions. This is exemplified in their Muir Torre syndrome (MTS) and its associated skin tumors, including sebaceous neoplasms and keratoacanthomas. MTS represents a subset of the hereditary non-polyposis colorectal carcinoma syndrome and is caused by mutations of genes encoding DNA mismatch repair proteins, including MLH1, MSH2, MSH3, MSH6, MLH3, PMS1 and PMS2.

MTS is inherited in an autosomal dominant manner with high penetrance and predisposes to a variety of malignancies, most commonly colorectal, but also endometrial, ovarian, genitourinary, and others. Importantly many of these malignancies tend to occur at an earlier age in MTS patients.

Diagnosis of MTS can be established based on skin findings alone. Detection of sebaceous neoplasms, including sebaceous adenoma (most common), sebaceous epithelium (sebaceoma) and sebaceous carcinoma, below the neck in a patient under 50 years of age, or in association with keratoacanthomas should raise suspicion for MTS. In addition, specific histologic features of sebaceous tumors, including keratoacanthoma-like or cystic changes, appear to be more commonly associated with MTS.

When MTS is suspected, immunohistochemical analysis of the skin neoplasm can be performed. Immunohistochemical stains are available for the proteins most commonly lost in MTS (MSH2, MLH1, PMS2 and MSH6) and a loss of two or more proteins has a high positive predictive value for the presence of MTS (up 100%).

Once the presence of MTS is confirmed, additional genetic testing is available. When the diagnosis has been established, preventive cancer screening programs of the patient and family members will facilitate early detection and treatment of other malignancies associated with MTS.

Further Reading

1. O. Abbas, M. Mahaling-ham: Cutanneous sebaceous neoplasmas as markers of Muir-Torre syndrome: a diagnostic algorithm. Journal of Cutaneous Pathology 2009; 36:613-619.

2. S. Shalin, S. Lyle, E. Calonje, A. Lazar: Sebaceous neoplasia and the Muri-Torre syndrome: important connections with clinical implications. Histopathology. 2010 Jan; 56(1): 133-47, Review.