Breast: Sezary Syndrome: A Unique Presentation.

Sezary syndrome is a subtype of cutaneous T cell lymphoma which usually presents as generalized skin disease with erytheroderma. Distal organ involvement is rare and is usually a late finding in the course of the disease. Breast involvement is extremely rare. Herein, we present a case report of a patient whose initial presentation involved an intramammary lymph node prior to the onset of more characteristic skin disease. Sezary syndrome was confirmed by cythopathologic findings.

Huntingtin interacting protein 1 as a histopathologic adjunct in the diagnosis of Merkel cell carcinoma.

BACKGROUND: Huntington interacting protein 1 (HIP1), an antiapoptotic protein normally expressed in the brain, is highly expressed in Merkel cell carcinomas (MCCs). Given this, the aim of the current study was to ascertain the value of HIP1 as a histopathologic adjunct in the diagnosis of MCC. METHODS: In this retrospective study, archival material from 26 cases with a diagnosis of MCC and/or neuroendocrine carcinoma were retrieved from the pathology files of the Skin Pathology Laboratory (Boston University School of Medicine, Boston, MA, USA). Histopathologic sections of all cases were re-reviewed and the diagnosis confirmed. All patient data were de-identified. Immunohistochemical studies were performed using antibodies to HIP1 and cytokeratins (CK) 20 and 7. RESULTS: A semiquantitative scoring system for immunohistochemical expression of HIP1 was utilized by deriving a cumulative score (based on percentage positivity of cells and intensity of expression). Using a cut-off total score of 3 or more as positive, the total number of positive cases was 22 for HIP1, 24 for CK20, and 11 for CK7. CONCLUSION: Comparing the results of HIP1 and CK20, there were four discordant pairs (three positive for CK20 but negative for HIP1 and one positive for HIP1 but negative for CK20). McNemar's test indicated that there was no statistical significance (P = 0.625), thereby implying a close agreement between the expression of HIP1 and CK20 in these neuroendocrine neoplasms.

Extraocular sebaceous carcinoma in situ: report of three cases and review of the literature.

Extraocular sebaceous carcinoma is a rare neoplasm. Purely in situ extraocular sebaceous carcinoma is extremely rare and somewhat controversial. Review of the literature reveals only three reported cases, two of which involved the head and neck and one the arm. The ones on the head and neck arose in association with an actinic keratosis. We report three cases of extraocular sebaceous carcinoma in situ and describe the first report of immunoperoxidase screening for mismatch repair proteins in such tumors.

Verruciform xanthoma of the upper-extremity in the absence of chronic skin disease or syndrome: a case report and review of the literature.

Verruciform xanthoma is a rare, benign lesion classically presenting on the oral mucosa or genital area. The etiology is not yet completely understood; however, verruciform xanthoma is often associated with (a) conditions of chronic inflammation or trauma, such as lichen sclerosis, recessive dystrophic epidermolysis bullosa, and pemphigus vulgaris, as well as in a setting of (b) chronic lymphedema, (c) chronic graft versus host disease, or (d) congenital epidermal nevi, such as those associated with the Congenital Hemidysplasia with Ichthyosiform nevus and Limb Defects (CHILD) syndrome. We report a case of a solitary verruciform xanthoma on the forearm of an 82-year-old man without history of chronic dystrophic skin disease or syndrome. In addition, a thorough literature review of extra-oral and extra-genital verruciform xanthomas is presented. On the basis of this review, we believe this case is an extremely rare presentation of a solitary verruciform xanthoma on the upper-extremity of an otherwise healthy individual.

Diagnostic utility of low-affinity nerve growth factor receptor (P 75) immunostaining in atypical fibroxanthoma.

BACKGROUND: Atypical fibroxanthoma (AFX) is a locally destructive, dermal-based, fibrohistiocytic, mesenchymal tumor. Immunohistochemistry helps to differentiate AFX from squamous cell carcinoma and spindle cell melanoma. Immunomarkers against p75 yield positive stains in spindled cell melanomas and negative stains in AFX, suggesting that these may be useful in differentiating these two entities. However, a recent study concluded that p75 is not a specific marker of neuroectodermal tumors; furthermore, p75 staining in AFX has only been evaluated in a few cases. METHODS: We stained 20 AFXs for p75 and various other markers. RESULTS: Reactivity was noted for vimentin (20 of 20 cases), CD10 (17/20), CD68 (14/20), CD99 (13/20), D2-40 (10/20) and p75 (1/20). CONCLUSIONS: We confirmed that CD99 and CD10 are frequently expressed in AFX (65 and 85%, respectively) and that CD31 rarely stains positive (5%). The 50% positivity rate of D2-40, in contrast with published evidence for its absence in melanoma, suggests that D2-40 may be useful for distinguishing AFX from melanoma. Furthermore, because only one sample was positive for p75, we confirm that p75 is useful in differentiating AFX from spindle cell melanoma. We advocate adding p75 and D2-40 to assist in differentiating AFX from melanoma.

Chromosomal copy number changes supporting the classification of lentiginous junctional melanoma of the elderly as a subtype of melanoma.

Recently the term lentiginous melanoma of the elderly has been suggested for a pattern of melanocytic neoplasia characterized by frequent occurrence in elderly patients, broad lentiginous growth pattern, with poorly cohesive nesting, suprabasilar extension of melanocytes and moderate cytological atypia. However, there are limited reported cases with follow-up information to confirm the malignant nature of these neoplasms. Using fluorescence in situ hybridization (FISH) targeting chromosomal loci that are frequently found to have copy number changes in melanoma, we evaluated cases of lentiginous junctional melanoma of the elderly in order to compare with the frequencies and patterns of chromosomal aberrations identified in other subtypes of melanoma. Previous studies have shown that by using a FISH assay targeting 6p25, 6q23, 11q13 and CEP6 with previously validated criteria, one could discriminate benign nevi from melanoma with high sensitivity and specificity. In this study, 16 of 19 cases (84%) showed sufficient copy number changes in one of the targeted chromosomal loci to meet FISH criteria for melanoma. A total of 17 control cases of lentiginous junctional nevi tested negative for all criteria. These findings support the classification of this pattern of melanocytic neoplasia as a subtype of melanoma.

Fluorescence in situ hybridization as a tool for microstaging in malignant melanoma.

Up to 30-50% of melanomas arise in association with a nevus. Accurately defining, the nevus from the melanoma can significantly affect microstaging. Recently, we showed that a targeted fluorescence in situ hybridization (FISH) assay could distinguish between benign nevi and melanoma with a sensitivity of 87% and specificity of 95%. In this study, we evaluated the potential of this same assay for use in the microstaging of melanoma. We performed FISH on 36 cases of melanoma occurring in association with a nevus and 6 cases of nevoid melanoma with deep dermal involvement. In the melanomas with associated benign nevi, FISH enumeration was performed separately on the histologically malignant and benign components. In the nevoid melanomas, FISH was performed on the deep dermal areas. On the basis of the criteria developed in our earlier studies, we determined the sensitivity of the assay within the malignant areas and the specificity within the benign areas of melanomas with associated nevi. In addition, we evaluated the sensitivity and specificity within a group of six nevoid melanomas with deep dermal involvement. Among melanomas with associated nevi, 28 of 36 cases (78%) tested positively in the histologically malignant areas. The benign nevus components were uniformly negative for all criteria. Six of six nevoid melanomas (100%) tested positively in the deep dermal area. FISH analysis with probes targeting 6p25, 6q23, 11q13 and CEP6 can effectively discriminate the malignant and benign components of melanomas with associated nevi and can be used as an adjunctive tool for microstaging. The assay has high sensitivity for the malignant areas of nevus-associated melanomas and outstanding specificity for the benign areas. The sensitivity is independent of the morphological features, and the assay performs well in nevoid melanoma cases.

Reactivation of dormant cutaneous Leishmania infection in a kidney transplant patient.

BACKGROUND: Leishmaniasis is an infection caused by a protozoan parasite belonging to genus Leishmania and transmitted by the Phlebotomus sandfly. Clinical presentations of infection include visceral, cutaneous, and mucocutaneous forms. Leishmaniasis is endemic in Africa, Asia, Europe, South America, and southern part of North America. This infection is extremely rare in the US and is mostly found among travelers coming from endemic areas. Cases of cutaneous and visceral leishmaniasis have been reported in organ transplant recipients in endemic areas. CASE REPORT: We describe a case of cutaneous leishmaniasis in a kidney transplant patient, originally from Bolivia, who resides in the area known to be non-endemic for leishmaniasis and who is known not to travel within or outside of the US after the transplantation. RESULTS: Histologic examination of cutaneous lesion revealed extensive subcutaneous lymphohistiocytic inflammation with clusters of amastigote within histiocytes. CONCLUSION: To our knowledge, this is the first case of cutaneous leishmaniasis in a kidney transplant patient residing in the US in an area known to be non-endemic for leishmaniasis, probably after reactivation of a previously dormant infection acquired outside of the US at least 9 months prior to developing clinical symptoms.

Primary carcinosarcoma of the helix of the ear.

We report our histologic and immunohistochemical findings in a rare case of cutaneous carcinosarcoma involving the helix of the ear. The tumor exhibited cellular features of both basal cell and squamous cell carcinoma and a malignant mesenchymal component that was consistent with malignant fibrous histiocytoma. The epithelial component exhibited a positive immunohistochemical reaction to cytokeratin and a negative reaction to vimentin, whereas the mesenchymal component showed a positive immunohistochemical reaction to vimentin and a negative reaction to cytokeratin. To the best of our knowledge, this is only the third reported case of a carcinosarcoma of the ear and the second case in which it developed on the helix.

'Atypical' and 'suspicious' diagnoses in breast aspiration cytology.

BACKGROUND: In 1996, the National Cancer Institute (NCI) recommended five categories for the diagnosis of breast aspiration cytology: benign, atypical, suspicious, malignant, and unsatisfactory. The authors evaluated the usefulness of separating inconclusive diagnoses into the aforementioned atypical and suspicious categories. The current study examined the correlation between cytologic and histologic findings made in breast aspiration cytology specimens that were categorized in accordance with these NCI guidelines. METHODS: From 1992 to 2000, 7727 breast aspirations were performed at the University of Miami/Jackson Memorial Medical Center (Miami, FL). Aspirates were classified using criteria similar to the NCI recommendations. Four hundred eighty-nine aspirates (6%) were classified as being cytologically 'atypical' or 'suspicious'. Of those, 225 'atypical' aspirates and 162 'suspicious' aspirates had available histologic follow-up data and were included in the study. RESULTS: Among the 'atypical' aspirates, 118 (52%) yielded malignant findings on histologic evaluation. Infiltrating ductal carcinoma (n = 87; 74%) was the most common malignant diagnosis, followed by infiltrating lobular carcinoma (n = 12; 10%). Among 'suspicious' aspirates, 135 (83%) yielded malignant findings on histologic analysis. The most common benign diagnosis in both 'atypical' and 'suspicious' aspirates was proliferative fibrocystic changes with or without atypia, followed by fibroadenoma. CONCLUSIONS: Most aspirates that yielded suspicious findings on cytologic examination proved to be malignant, as did > 50% of aspirates that yielded atypical findings. Infiltrating lobular carcinoma commonly was underdiagnosed as being atypical on cytologic examination. Benign lesions with atypical or suspicious cytologic diagnoses included certain cases of atypical ductal hyperplasia. The authors concluded that the distinction between the atypical and suspicious categories, as recommended by the NCI, is not warranted. Therefore, they suggest the use of a single term, such as 'equivocal', to describe inconclusive diagnoses on breast fine-needle aspiration cytology.

Lymphomatoid papulosis in an HIV-positive man.

Lymphomatoid papulosis (LyP) is a rare cutaneous lymphoproliferative condition characterized by a chronic, recurrent eruption of papules and nodules that undergo spontaneous regression. The disorder is usually clinically benign; with a minority of cases progressing to malignant lymphoma. LyP is divided into two subtypes based on histologic appearance. Type A resembles Hodgkin's disease with up to 20% of large CD30+ lymphocytes. Type B resembles mycosis fungoides showing an infiltrate of CD4+ lymphocytes and scattered CD30+ cells. Clinically LyP often resembles pityriasis lichenoides et varioliformis acuta but has a strikingly different histological appearance. Histologically, LyP resembles lymphoma (anaplastic T-cell or Hodgkin's) but is distinguished by its benign course. Here we present a case of LyP in a severely immune-repressed HIV-positive patient. This patient presented with pruritic papules involving the upper extremities and a CD4+ T-cell count of 4. Histopathologic examination showed a dense superficial dermal infiltrate comprising normal-sized lymphocytes admixed with larger lymphocytes. Immunophenotyping showed most of the lymphocytes to be CD3+ (T cells). The scattered larger cells were CD30+. The smaller lymphocytes were CD8+ rather than CD4+ as expected for non-HIV-appointed LyP. This may be because of the immune disregulation of HIV disease and the absolute and relative paucity of CD4+ T cells relative to CD8+ T cells.