Mark A. Hurt

Granular cell atypical fibroxanthoma

Abstract:  We report on two patients with granular cell atypical fibroxanthoma. Both neoplasms were solitary, light‐tan, dome‐shaped papules on sun‐exposed areas of the head in two elderly white men. Microscopically, these neoplasms showed a dermal proliferation of pleomorphic granular cells with irregular hyperchromatic nuclei, multinucleated cells, and scattered mitoses. Immunohistochemical stains were positive for CD68 and vimentin and negative for Melan‐A or human melanoma black (HMB)‐45, S‐100 protein, pancytokeratin, and actin, consistent with atypical fibroxanthoma. The differential diagnosis of granular cells in neoplasms containing cytological pleomorphism is challenging in view of the many different neoplasms that may present with granular cytoplasm. These include the conventional granular cell tumor and its malignant form, leiomyoma, leiomyosarcoma, dermatofibroma, dermatofibrosarcoma protuberans, and angiosarcoma.

Syringomatous squamous tumors of the breast

Four cases are reported of syringomatous squamous tumors of the breast occurring in women aged 37 to 70 years. The lesions were characterized histologically by relatively well‐circumscribed tumor‐like nodules composed of a proliferation of teardrop or comma‐shaped islands of squamous epithelium. The squamous epithelial islands contained central lumens lined by eosinophilic cuticles and were surrounded by a densely cellular fibrous matrix, thus closely resembling the growth pattern of dermal eccrine syringomas. The lesions appeared to arise de novo from breast parenchyma without evidence of transitions with the surrounding normal or hyperplastic mammary epithelium and were not associated with the overlying skin or nipple epidermis. In all cases, the surrounding breast tissue showed fibrocystic and benign proliferative changes, and in one case, the lesion was found in the vicinity of a large cyst surrounded by microcalcifications. All patients were treated by local surgical excision and have shown no evidence of recurrence over a follow‐up period of 1 to 6 years. The histologic differential diagnosis and the possible pathogenesis of these lesions are discussed.

Adenolipoma of the skin: A report of nine cases

BACKGROUND We have identified nine cases of a variant of superficial lipoma that are characterized by the presence of eccrine sweat glands. OBJECTIVE The purpose of this study was to elucidate the clinical and histopathologic features of this distinctive lesion. METHODS Nine cases of lipoma that contain eccrine glands were retrieved from our pathology files from 1989 through 1992. Their clinical and histopathologic features were studied and compared with those of conventional lipoma. RESULTS The clinical features of these lesions did not deviate appreciably from those of the usual lipomas. However, their microscopic appearances differed by the presence of displaced and distorted eccrine glands. One lesion had the added features of a myxolipoma. CONCLUSION Adenolipoma of the skin is a distinct lesion that can occur in the dermis or subcutaneous tissue.

EPIDERMOTROPIC NEUROENDOCRINE CARCINOMA : IMMUNOHISTOCHEMICAL DIFFERENTIATION FROM SIMULATORS, INCLUDING MALIGNANT MELANOMA

Epidermotropic neuroendocrine carcinoma (NEC) is rare. Based on such a case in an 88‐year‐old woman with a facial NEC showing epidermotropism with a pagetoid growth pattern, we asked whether several similar tumors involving the epidermis could be easily differentiated by immunohistochemical methods. We constructed a panel of control cases (2 each) for NEC, clear cell Bowens disease (CCBD), Pagets disease (PD), superficial basal cell carcinoma (SBCC), cutaneous T‐cell lymphoma (CTTL), and superficial spreading malignant melanoma (SSMM) to compare with our patient. A panel of antibodies including epithelial membrane antigen (EMA), neuron specific enolase (NSE), AE1/3 cytokeratin (CK), carcinoembryonic antigen (CEA), leukocyte common antigen (LCA), S‐100, and HMB‐45 were applied. Cutaneous NEC controls and our patients tumor were strongly positive for EMA and NSE and had paranuclear dot‐like cytoplasmic positivity for CK. CCBD was moderate to strong for CK. PD was strong for CEA. SBCC was essentially negative for all. CTTL was strong for LCA. SSMM was strong for S‐100 and HMB‐45. Controls were either negative or weak for the antibodies not mentioned. We conclude that this antibody panel can reliably differentiate these epidermotropic or juxtaepidermal tumors in diagnostic dermatopatholpgy and should be applied to lesions requiring separation beyond H&E capabilities, especially with superficial shave biopsies showing small cell “Pagetoid” growth patterns.

Bullous lesions in acrodermatitis enteropathica delaying diagnosis of zinc deficiency : a report of two cases and review of the literature

Acrodermatitis enteropathica (AE) is a rare disorder associated with poor absorption of zinc. A variety of clinical and histological findings have been reported in the literature, described mainly in isolated case reports. Because of the varied nature of these cases, the histological features of AE are described often as non‐specific. We describe lesions of AE in two patients who presented with vesiculobullous and erosive skin lesions, both showing intra‐epidermal, inflammatory vesiculation with surrounding eosinophilic epidermis and necrotic keratinocytes. The lack of clinical suspicion of AE led to their misdiagnosis. We present these two patients to further characterize the bullous variant of AE, and we review the previously reported clinical and histopathological findings.

Keratoacanthoma vs. squamous cell carcinoma in contrast with keratoacanthoma is squamous cell carcinoma

To the Editor Forslund et al. accept the premise that keratoacanthoma (KA) and cutaneous squamous cell carcinoma (SCC) are not within the same biological and diagnostic spectrum, in contrast with recent thinking by a number of authors, notably Ackerman, who regard solitary KA as a specific type of SCC. Forslund et al. state that ‘Keratoacanthomas are benign, clinically distinct skin tumors generally located at sun-exposed sites in elderly fair-skinned people. . .Histologically, keratoacanthomas are diagnosed by their architecture as well as their cytological features, and when also considering their characteristic clinical history most keratoacanthomas can be distinguished from SCCs.’ The authors do not state whether they believe KAs are examples of hyperplasia or neoplasia. This distinction, however, is important because hyperplasias are reversible conditions, whereas neoplasms, as a rule, do not resolve spontaneously. To approach this issue another way, if one were to develop the hypothesis that KA is hyperplasia, rather than neoplasia of keratocytes, the finding of human papillomavirus (HPV) is an intriguing premise on which to base that investigation, as any dermatologist or dermatopathologist knows from dealing with verrucae vulgares. In order to investigate that question scientifically, one would, however, need to identify a control group of uncontroversial squamous carcinomas as part of any such study, in addition to the experimental group, i.e. KAs. Further-more, it would also be extremely useful to find examples of ‘KAs’ that metastasized (i.e. unequivocal SCCs that are well differentiated) and to subject these lesions to a study of a possible association with HPV. In the study by Forslund et al., however, I do not see any evidence that they considered investigating controversial or uncontroversial cutaneous SCCs as a control group. Thus, how are the authors to know whether HPV might be detected in cutaneous SCCs? Furthermore, how would the authors respond to the advocates of KAs are squamous carcinomas? Those who include KAs as examples of SCCs would argue that some SCCs contain HPV, and, perhaps, that HPV is an inducer, not unlike HPV 16and 18-associated SCCs that have been documented well in the literature. They might also argue it that it is not causal at all – true, true but unrelated. In contrast, the argument from those who believe that KA is hyperplasia would probably favor the point of view that HPV causes KA, which regresses once the effects of HPV have played out. However, without using control groups of welldocumented examples that either camp would agree are KAs or SCCs, the presence of HPV in a KA has no scientific meaning whatsoever, at least regarding the question of KA vs. SCC in contrast with KA is SCC. Finally, the authors did not show examples photographically of what they were investigating, thus the reader cannot tell whether these are classical KAs or classical SCCs. I urge the authors to clarify their nosological position on KA and to show photographically what they mean by the use of that term. Yours sincerely

Fibroepithelioma-like changes associated with anogenital epidermotropic mucinous carcinoma. Fibroepitheliomatous Paget phenomenon.

We describe two patients with crusted perincal plaques that were biopsied and diagnosed as Pagets disease. Resection specimens of each contained a dermal mucinous carcinoma with extensive epidermotropism and coexistent epidermal basaloid proliferations closely resembling fibroepithelioma (Pinkus). The presence of the Paget phenomenon was supported by histochemical, immnunohistochemical, and ultrastructrual evidence. No other primary neoplasms were found in either patient. Followup at 2 1/2 and 3 1/2 years, respectively, has been negative. We conclude that either the fibroepitheliomatous changes may be induced by or may represent a collision (unlikely) with the epidermotropic mucinous carcinoma. It is proposed that the concept fibroepitheliomatous Paget phenomenon be used to stand for the histologic changes common to our cases as well as those previously reported.

Types of melanoma

T here is, perhaps, no other class of neoplasm in the discipline of cutaneous medicine that evokes fear in patients as deeply as does melanoma. This sarcoma, seemingly unpredictable as it is in its clinical presentation and its histologic mimicry of melanocytic nevi and other lineages of neoplasms, is indeed worthy of our respectebut not our fear. Fear is overcome by knowledge, and methods of diagnosis and management are its consequence. The last 60 years has been a period of focused research by dermatologists and dermatopathologists toward the desideratum of understanding which criteria, either alone or in combination, aid in establishing the diagnosis of melanoma. There has been a small, but intellectually potent, group of thinkers and writers who, during this period, have attempted to discover the criteria that dermatologists and dermatopathologists use to diagnose and report melanomas daily throughout the world. The most notable among these are Spitz, Allen, Helwig, Reed, Clark, Mihm, Ackerman, Elder, Breslow, Barnhill, and LeBoit. Yet, these same authors, the developers of the language of melanoma, disagree often with each other about the meanings of the words applied to stand for different patterns of those dreaded neoplasms. One person’s ‘‘superficial spreading melanoma’’ is another person’s ‘‘lentiginous melanoma.’’ ‘‘Nodular melanomas’’ for some are simply ‘‘nodules of melanoma’’ for others. One person’s ‘‘vertical growth phase’’ is another person’s refusal to accept such language on the grounds that no one knows

The melanocytic nevus described by Clark et al. What is its nature? What should it be named? An answer from history and from logic.

The article by Shapiro et al., which summarizes a survey of respondents on the words or phrases they use to identify the melanocytic nevus described by Clark et al. in 1978, shows that over 70% of The American Society of Dermatopathology (ASDP) members and nearly 85% of The American Academy of Dermatology (AAD) members preferred one of the following phrases: ‘‘dysplastic nevus’’, ‘‘nevus with architectural disorder’’, ‘‘atypical nevus’’, or ‘‘atypical melanocytic hyperplasia’’. Only about 11% of the ASDPmembers and less than 5% of the AAD members identified the nevus as either a ‘‘Clark’s nevus’’ or a ‘‘compound nevus’’, thereby avoiding the designations ‘‘dysplastic’’, ‘‘architectural disorder’’, and ‘‘atypical’’. This article revealed one important fact about the nature of the melanocytic nevus that has for over 26 years eluded and confounded dermatopathologists, pathologists, and dermatologists alike. The fact is that the disparate, inconsistent nomenclature applied to this particular type of melanocytic nevus reflects a conceptual conundrum that exists in the minds of susceptible histopathologists. This nomenclature results in producing confusion and uncertainty and implies that melanoma cannot be excluded in these lesions vis-a-vis the pairing of the terms ‘‘atypical’’, ‘‘dysplastic’’, or ‘‘disordered’’ (implying that one cannot exclude malignancy) with ‘‘melanocytic nevus’’ (a phrase of certainty that refers to a hamartoma or benign neoplasm). The actual melanocytic nevus in question is, however, either a hamartoma or a benign neoplasm that never killed anyone and never will. Its diagnosis should, therefore, be clean, clear, and unequivocal. What is the origin of this uncertainty? Why have 26 years, two consensus conferences (resulting in no consensus), and tens of millions of dollars of National Institutes of Health’s grant money failed to produce a rational, uniform approach to the diagnosis of this type of melanocytic nevus? In my opinion, there are several reasons for this failure, chief among which are:

Sclerotic Glomus Tumor

We report an unusual histopathological variant of a glomus tumor that arose in a peculiar topographic site, a sclerotic glomus tumor. Unlike conventional glomus tumors or glomangiomas that have a loose fibrous stroma with variable hyaline and myxoid changes, the case reported herein had a diffuse, hyalinized, sclerotic stroma. A further difference was that the majority of glomus tumors and glomangiomas occur in the subungual area, trunk, or extremities, whereas the present tumor occurred on the ear. Due to the peculiar histological features and location, other tumors were considered in the differential diagnosis to include Merkel cell carcinoma, primitive neuroectodermal tumor, and small cell melanoma. This article illustrates a unique variant of a glomus tumor, which to our knowledge has not been previously described.