Alistair Robson

Eccrine porocarcinoma (malignant eccrine poroma): a clinicopathologic study of 69 cases.

The clinicopathologic characteristics of 69 cases of eccrine porocarcinoma (EP) have been studied. Seven cases of purely in situ disease are included. Forty patients were female, 29 male with ages ranging from 29 to 91 years (mean 73 years). The lower extremity represented the single most common site (44%). Other common sites were the trunk (15 cases, 24%) and head (11 cases, 18%). The histologic diagnosis of EP was predicated on the basis of an irregular tumor at least partly formed of characteristic poromatous basaloid epithelial cells displaying ductal differentiation, and significant cytologic atypia. Forty-seven tumors (68%) contained mature well-formed eccrine ducts having an eosinophilic luminal cuticle, with the remaining tumors containing small ill-formed ducts and/or intracytoplasmic lumina. All ducts were discernible via light microscopy and in 49 cases were highlighted with DPAS stain and/or CEA/EMA immunocytochemistry. A variant with a broad pushing tumor margin and marked nuclear pleomorphism showed some resemblance to proliferative bowenoid dysplasia. In 11 cases (18%) the tumors appeared to arise in continuity with a benign preexistent poroma. A variety of histologic patterns were displayed including clear, squamous, and spindle cell differentiation, mucus cell metaplasia, and colonization by melanocytes. Lymphovascular invasion was present in 9 cases (15%). Three cases showed pagetoid extension of malignant cells (epidermotropism) and appeared to be multifocal. Follow-up was available in 54 patients (78%) with 9 (17%) experiencing local recurrence, 10 developing lymph node metastases (19%), and 6 (11%) experiencing distant metastases or death. Mitoses, the presence of lymphovascular invasion, and tumor depth >7 mm were associated with a poorer prognosis. Dividing tumors into those with a “pushing” or “infiltrating” advancing margin was also predictive of outcome with the latter having an increased risk of local recurrence. This report, the largest series of EP to date, suggests that the incidence of aggressive behavior is less than popularly believed. Furthermore, EP can display a wide variety of histologic patterns that may lead to diagnostic error in the unwary. The large number of cases in this series enables a reliable evaluation of prognostic parameters. A more aggressive clinical course may be indicated by more than 14 mitoses per high power field (hazard ratio [HR] for death 17.0, 95% confidence interval [CI] 2.71–107), lymphovascular invasion by tumor (HR 4.41, CI 1.13–17.2), and depth >7 mm (HR 5.49, CI 1.0–30.3). Thus, mitoses, lymphovascular invasion, and tumor depth should be evaluated in these tumors. We also suggest that tumors presenting an “infiltrative” advancing margin are particularly prone to local recurrence and require wide excision with close attention to the surgical margins by the reporting pathologist.

Granulomatous mycosis fungoides and granulomatous slack skin: a multicenter study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization For Research and Treatment of Cancer (EORTC)

BACKGROUNDnGranulomatous cutaneous T-cell lymphomas (CTCLs) are rare and represent a diagnostic challenge. Only limited data on the clinicopathological and prognostic features of granulomatous CTCLs are available. We studied 19 patients with granulomatous CTCLs to further characterize the clinicopathological, therapeutic, and prognostic features.nnnOBSERVATIONSnThe group included 15 patients with granulomatous mycosis fungoides (GMF) and 4 with granulomatous slack skin (GSS) defined according to the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas. Patients with GMF and GSS displayed overlapping histologic features and differed only clinically by the development of bulky skin folds in GSS. Histologically, epidermotropism of lymphocytes was not a prominent feature and was absent in 9 of 19 cases (47%). Stable or progressive disease was observed in most patients despite various treatment modalities. Extracutaneous spread occurred in 5 of 19 patients (26%), second lymphoid neoplasms developed in 4 of 19 patients (21%), and 6 of 19 patients (32%) died of their disease. Disease-specific 5-year survival rate in GMF was 66%.nnnCONCLUSIONSnThere are clinical differences between GMF and GSS, but they show overlapping histologic findings and therefore cannot be discriminated by histologic examination alone. Development of hanging skin folds is restricted to the intertriginous body regions. Granulomatous CTCLs show a therapy-resistant, slowly progressive course. The prognosis of GMF appears worse than that of classic nongranulomatous mycosis fungoides.

Primary cutaneous apocrine carcinoma: A clinico-pathologic analysis of 24 cases

Primary cutaneous apocrine carcinoma is a rare malignancy. This study of 24 examples suggests that the prognosis is not always poor and that grading criteria devised for breast carcinoma may have utility in this group of malignancies. Furthermore, steroid receptor expression should be investigated in these tumors, particularly if a tumor is unlikely to be controlled by surgery alone.

A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome

BACKGROUNDnThere is no prognostic index for primary cutaneous T-cell lymphomas such as mycosis fungoides (MF) and Sezary syndrome (SS).nnnMETHODnTwo prognostic indices were developed for early (IA-IIA) and late stage (IIB-IVB) disease based on multivariate data from 1502 patients. End-points included overall survival (OS) and progression free survival (PFS). External validation included 1221 patients.nnnFINDINGSnSignificant adverse prognostic factors at diagnosis consisted of male gender, age >60, plaques, folliculotropic disease and stage N1/Nx for early stage, and male gender, age >60, stages B1/B2, N2/3 and visceral involvement for late stage disease. Using these variables we constructed two separate models each defined using 3 distinct groups for early and late stage patients: 0-1 (low risk), 2 (intermediate risk), and 3-5 factors (high risk). 10 year OS in the early stage model was 90.3% (low), 76.2% (intermediate) and 48.9% (high) and for the late stage model 53.2% (low), 19.8% (intermediate) and 15.0% (high). For the validation set significant differences in OS and PFS in early stage patients (both p<0.001) were also noted. In late stage patients, only OS differed between the groups (p=0.002).nnnINTERPRETATIONnThis proposed cutaneous lymphoma prognostic index provides a model for prediction of OS in early and late stage MF/SS enabling rational therapeutic choices and patient stratification in clinical trials.

Management of cutaneous squamous cell carcinoma in patients with epidermolysis bullosa: best clinical practice guidelines

This article summarizes recommendations reached following a systematic literature review and expert consensus on the diagnosis and management of cutaneous squamous cell carcinomas in people with epidermolysis bullosa. The guidelines are intended to help inform decision making by clinicians dealing with this complex complication of a devastating disease.

Indolent CD8-positive lymphoid proliferation of acral sites: three further cases of a rare entity and an update on a unique patient.

Primary cutaneous indolent CD8‐positive lymphoid proliferation is an emerging entity characterized by slowly enlarging papules and nodules that are pathologically comprised of clonal nonepidermotropic medium‐sized atypical CD8(+) T‐cells. Although the majority of lesions are solitary and located on the ears, bilateral symmetrical presentations have been described and lesions may arise at other peripheral or ‘acral’ sites. Patients follow a benign clinical course and systemic involvement has not yet been observed. Despite this, some medical practitioners classify such lesions as peripheral T‐cell lymphoma, NOS, a category implying aggressive disease.

Early-onset dermatosis papulosa nigra.

tumour mass and location can impede both afferent and efferent signalling and, as a measure of (over)compensation, lead to a disturbed sympathetic nerve response on the contralateral side. In this case we believe that amplified sympathetic innervation led to localized hyperhidrosis and low palmar temperature, the latter caused by vasoconstriction. The autonomic nervous system is complex and the mechanisms leading to the patient’s symptoms are not completely elucidated. Contralateral segmental hyperhidrosis and temperature difference have previously been reported due to adenocarcinoma of the lung with costal invasion. Another report describes the case of an apical pulmonary adenocarcinoma with contralateral localized hyperhidrosis. Other studies and medical cases offer mechanistic insight. Following unilateral thoracic sympathectomy (T3) in patients with palmar hyperhidrosis, the contralateral palmar skin temperature drops temporarily, suggesting the existence of a cross-inhibitory effect by postganglionic neurons, not only at the level of the upper extremities but also between the upper and lower extremities. The latter also offers an explanation for the compensatory hyperhidrosis observed in some patients as an undesirable side-effect after sympathectomy. However, these studies do not offer an explanation for the different segmental localizations of axillary hyperhidrosis and palmar hypothermia. It has been shown that sympathetic skin nerve activity induced by cooling can consist of vasoconstrictor impulses exclusively. An alternative explanation is the pressure caused by the tumour on the sympathetic ganglia, and rami communicantes affecting some nerves more than others. In conclusion, this case illustrates an interesting yet unexplained phenomenon. We cannot offer proof that the tumour antedates the symptoms. Neither did we test the function of the vasoconstrictor and sudomotor function of the extremities through electrodermal and plethysmographic recordings, which would have provided valuable information. This case shows a probable causal relationship between segmental partial affection of the sympathetic nervous system (unilateral localized hyperhidrosis and hypothermia) to a contralateral tumour, rather than a coincidental association between two rare disorders. The case also illustrates the importance to recognize and report cutaneous symptoms as possibly paraneoplastic.

Follicular proliferation or basal cell carcinoma? The first prospective U.K. study of this histological challenge during Mohs surgery

DEAR EDITOR, Mohs micrographic surgery (MMS) provides the highest cure rate for the treatment of basal cell carcinoma (BCC) while maximizing tissue conservation. Key to the success of MMS is the correct diagnosis of the tumour. However, the facial area has a high density of follicular and sebaceous epithelia, which can be difficult to distinguish from BCC. Folliculocentric basaloid proliferation (FBP) is a term that attempts to define these abnormal follicular entities, sometimes misdiagnosed as BCC. This was first described in a group of patients undergoing MMS for nasal and perinasal BCC; however, the frequency is unreported. We prospectively identified 18 cases of challenging benign follicular proliferations from 800 patients undergoing MMS for facial BCC between June 2012 and December 2013. Patients were identified during surgery by an experienced Mohs surgeon and later confirmed by a second Mohs surgeon and an expert dermatopathologist. In our practice we use haematoxylin and eosin staining, and the interval between our histological sections is 50–100 lm. Histologically these follicular proliferations met the criteria for FBP. The frequency of FBP was found to be 2 3% (18 of 800 patients) of all cases of MMS for facial BCCs. Thirteen of 18 (72%) were female and the average age was 69 years (range 43–86). Fifteen of the 18 patients (83%) had primary BCC and the remainder had recurrent lesions. The average number of stages required was 1 9 0 58 (range 1–4). Fourteen cases involved surgery in the nasal area (five nasal tip, five nasal side wall, four nasal ala); the remaining cases involved the temple, lower eyelid, forehead and ear. Figure 1 is an example of these challenging cases and demonstrates benign follicular proliferations. These cases illustrate the difficulty in distinguishing FBP from BCC. FBP was first described by Leshin and White in 1990 and is characterized by a multishaped, multifocal basaloid proliferation that frequently involves the follicular epithelium. This entity commonly appears in aggregates, with uniform individual cells that can have peripheral palisading. The original report described 22 cases undergoing MMS for nasal or perinasal BCC, and notably, in keeping with this finding, the majority of our cases also involved the nasal area. All patients (a)

Pityriasis rubra pilaris with histologic features of lichen nitidus

Fig 2. Pityriasis rubra pilaris (case 1). Parakeratosis and psoriasiform acanthosis with a light perivascular lymphocytic infiltrate of the dermis. There is lichenoid inflammation with a granulomatous component, reminiscent of lichen nitidus. (Hematoxylin-eosin stain; original magnification 340.) REFERENCES 1. Wilkin JK. Rosacea: pathophysiology and treatment. Arch Dermatol. 1994;130:359-362. 2. Wilkin J, Dahl M, Detmar M, et al, National Rosacea Society Expert Committee. Standard grading system for rosacea: report of the National Rosacea Society Expert Committee on the classification and staging of rosacea. J Am Acad Dermatol. 2004;50:907-912. 3. Lee YB, Shin JY, Cheon MS, Oh ST, Cho BK, Park HJ. Photorejuvenation using long-pulsed alexandrite and long-pulsed neodymium:yttrium-aluminum-garnet lasers: a pilot study of clinical outcome and patients’ satisfaction in Koreans. J Dermatol. 2012;39:425-429. 4. Taylor MB, Prokopenko I. Split-face comparison of radiofrequency versus long-pulse Nd-YAG treatment of facial laxity. J Cosmet Laser Ther. 2006;8:17-22. 5. Mahmoud BH, Tierney E, Hexsel CL, Pui J, Ozoq DM, Hamzavi IH. Prospective controlled clinical and histopathologic study of hidradenitis suppurativa treated with the long-pulsed neodymium:yttrium-aluminium-garnet laser. J Am Acad Dermatol. 2010;62:637-645.