Spitz nevi demonstrate a diverse spectrum of morphologies. Recently, there have been two reported examples of Spitz nevi with rosette-like structures similar to Homer-Wright rosettes. Rosettes have also been described in melanomas and in a proliferative nodule arising in a congenital nevus.
A retrospective review of 104 cases of Spitz nevi and variants (n = 51), pigmented spindle cell nevi (n = 26), combined melanocytic nevi with features of Spitz (n = 8), atypical Spitz tumor (AST, n = 9), and spitzoid melanoma (n = 10).
Rosette-like structures were present in 3 of the 104 cases (2.9%), including a compound Spitz nevus, a desmoplastic Spitz nevus, and an AST. All three cases demonstrated several foci of small nests of epithelioid cells with peripherally palisaded nuclei arranged around a central area of fibrillar eosinophilic cytoplasm. Immunohistochemical staining of the three spitzoid lesions demonstrated that the rosette-like structures express S100 protein, Melan-A, and neuron specific enolase (NSE) and lacked expression of neurofilament, glial fibrillary acidic protein, and synaptophysin.
While uncommon, rosette-like structures can occur as a focal feature in Spitz nevi and AST. Rosette-like structures may represent a normal morphologic finding in Spitz nevi, and awareness of them may prevent misdiagnosis as a neural tumor or melanoma.
Sclerotic bodies are round to oval structures made up of collagen with entrapped elastic fibers, which may be sometimes ossified. These bodies may be found in skin biopsies from patients with nephrogenic systemic fibrosis (NSF), a disease linked to the use of gadolinium in radiologic procedures and chronic renal failure. Sclerotic bodies have not been reported in other diseases. Herein, we report sclerotic bodies as an incidental finding in a re-excision specimen of a squamous cell carcinoma (SCC) from the forearm of an 85 year old man with chronic renal failure. The patient had had multiple SCC removed over time. Additional clinical history revealed patient having received gadolinium in 2003 and 2004, preceding his dialysis that began in 2009. All of his excision specimens revealed sclerotic bodies in 20 of 30 specimens beginning in 2008. None of the 26 re-excision specimens prior to gadolinium exposure had these bodies. Our findings suggest that sclerotic bodies are the result of gadolinium exposure in patients with chronic renal insufficiency. Because the bodies were found near the re-excision scar, it may be that gadolinium or its metabolites activate fibroblasts in the setting of wound healing. The reasons why this patient did not develop NSF are unclear.
Virtual microscopy is increasingly being used in dermatopathology educational settings.
To evaluate diagnostic accuracy and attitudes between virtual microscopy and traditional glass slide microscopy among dermatology residents.
A 48-question dermatopathology examination was administered to thirty-five dermatology residents at 3 different dermatology residency training sites during the 2011-2012 academic year with half (n = 24) of the questions using the gold standard of glass slide microscopy and half (n = 24) using whole, scanned virtual slides. Correct number of questions using glass slides and virtual slides was evaluated. Participants were surveyed regarding previous experience with digital slide imaging; quality, ease of use, and speed of slide review; and overall microscopy preferences.
Overall, diagnostic accuracy was better with glass slides than virtual slides (P = .01). However, no statistically significant difference was found in diagnostic accuracy of first-year trainees (P > .99) or trainees with exposure to virtual microscopy greater than 2 times per month (P = .27). There was no overall personal preference for glass slide vs virtual microscopy.
Different cases and questions were used for glass slides and virtual microscopy.
Diagnostic accuracy with virtual microscopy is dependent on year of residency training and prior experience with virtual microscopy.
Desmoplastic melanoma can be difficult to distinguish from desmoplastic melanocytic nevi both clinically and histopathologically. Several attempts have been made to explore the use of ancillary studies to facilitate this distinction. Prior work has suggested that immunohistochemical expression of p16 could help distinguish sclerosing Spitz nevi from desmoplastic melanomas. We re-evaluated the expression of p16 in 22 desmoplastic melanomas (thirteen mixed and nine pure desmoplastic tumors) and five desmoplastic melanocytic nevi (three desmoplastic Spitz nevi and two congenital melanocytic nevi with prominent dermal sclerosis). All desmoplastic melanocytic nevi were strongly immunoreactive for p16. Of the 22 desmoplastic melanomas, six tumors failed to label for p16, ten were focally positive, but six tumors were diffusely immunoreactive. The latter finding is relevant, since it points to limitations in the diagnostic value of immunohistochemical staining for p16 for the diagnosis of desmoplastic melanocytic proliferations. Diffuse staining for p16 is not restricted to desmoplastic Spitz nevi but can also occur in a subset of desmoplastic melanomas, and this warrants caution in the use of this marker for diagnostic purposes.
Diffuse dermal angiomatosis (DDA) represents a benign, acquired, reactive proliferation of vessels. DDA is clinically characterized by painful livedoid plaques with central ulceration, and the histopathologic hallmark is diffuse endothelial cell hyperplasia in the dermis. DDA has been rarely reported in association with calciphylaxis, a condition characterized by calcification of arterial walls with accompanying thrombosis and cutaneous necrosis. We present a case of a 72-year-old man with end stage renal disease on peritoneal dialysis who presented with painful lesions on his legs, and was found to have DDA in the setting of calciphylaxis. The possible pathogenesis linking DDA and calciphylaxis is discussed.
We sought to describe expression of the immunohistochemical markers, CD31, D2-40 and LYVE-1, that may assist in differentiating in cutaneous lymphangioma and cutaneous hemangioma
Immunohistochemical staining of lymphangioma and hemangioma specimens was completed with CD31, D2-40 and LYVE-1 antibodies.
33 examples of lymphangioma and 13 examples of hemangioma were evaluated. CD31 was positive in 91% of lymphangioma and 100% of hemangioma (sensitivity 0.91, 95% confidence interval 0.75-0.98; specificity 0, 95% confidence interval 0-0.28). D2-40 was positive in 54% of lymphangioma and 23% in hemangioma (sensitivity 0.50 95% confidence interval 0.32-0.68; specificity 0.50 95% confidence interval 0.28-0.72). LYVE-1 was positive in 6% in lymphangioma and 15% hemangioma (sensitivity 0.06 95% confidence interval 0.01-0.22, specificity 0.85 95% confidence interval 0.54-0.97).
Due to the similar embryonic origins, it is unlikely that a single marker may accurately differentiate the lymphangioma from hemangioma. A combination of immunohistochemical stains such CD31, D2-40 and LYVE-1 testing may potentially increase the diagnostic accuracy in differentiating lymphangioma from hemangioma. However, our findings suggest that there is no advantage in performing immunohistochemical staining to differentiate lymphangioma from hemangioma.
Matricoma is benign follicular neoplasm with the same constituent cells as pilomatricoma but with a different silhouette.1 Matricoma consists mostly of solid aggregations of matrical cells, as opposed to pilomatricoma, which is often a cystic lesion. We herein describe a case of agminated pigmented matricoma. A 27-year-old Japanese man visited our hospital complaining of three separate, pigmented, firm, sessile nodules on the back. Histopathologic examination revealed mostly solid aggregations of matrical and supramatrical cells located mainly within the dermis and the upper part of the subcutis. Shadow cells were present mostly within the aggregations. At higher magnifications, occasional indication of inner sheath differentiation, in addition to differentiation toward hair in the form of shadow cells, could be discerned in the center of the aggregations. The matrical and supramatrical cells within the aggregations were relatively uniform in size and shape, and contained several mitotic figures. Heavily pigmented melanocytes were present within the aggregations of matrical and supramatrical cells, and heavily pigmented melanophages were found in adjacent stroma. Pigmentation is a rare event in benign and malignant adnexal tumors, and to our knowledge, no similar case has been reported.
Lichen planus follicularis tumidus (LPFT) represents an uncommon variety of lichen planus (LP). Clinically, it presents with prominent purplish lesions or white-pigmented yellowish cysts and comedones. Histopathologically, it is similar to lichen planopilaris, and it is additionally characterized by follicles and cysts surrounded by a lichenoid lymphocytic infiltrate. The most common location is the retroauricular region, and it may be associated with other variants of LP. Herein, we describe the case of a 50-year-old woman with a history of lower limb hypertrophic LP who subsequently presented with multiple pink, tumid, pruritic plaques with white-yellow cysts and comedones extensively affecting the bilateral face. Histopathologic examination revealed a lichenoid infiltrate surrounding the follicles and cysts. We diagnosed LPFT and began treatment with topical corticosteroids, antihistamines, systemic corticosteroids, and oral acitretin without improvement. Subsequently, the patient had an acceptable response to cyclosporine at doses of 5 mg/kg/day with remission of itching and tumidity but with residual cysts and comedones remaining. To date, the literature contains only 16 cases of LPFT. To our knowledge, this is the most severe case and is the only one with cessation of disease activity in response to cyclosporine.
Antimalarials are commonly prescribed in medical practice for conditions such as rheumatoid arthritis, lupus erythematosus, as well as malaria. They are generally well-tolerated, but side effects, although infrequent, are well known. The antimalarial chloroquine diphosphate may be associated with a bluish-gray to black hyperpigmentation of the oral mucosa, mainly on the hard palate. In this report we described five additional cases of palate hyperpigmentation related to the chronic use of chloroquine diphosphate. Professionals must be aware of the adverse effects of antimalarials as chloroquine diphosphate in order to make the correct diagnosis and appropriate management of the patient. Early diagnosis of oral pigmentation by antimalarials may be of great relevance, because it might be an early sign of ocular involvement, and therefore it may be helpful to prevent further complications of antimalarial therapy for the patient.
]]>Solitary epidermolytic acanthoma is thought to be an uncommon lesion. It can present as a solitary, localized or disseminated process that is unrelated to the genetic form of icthyosis.
A retrospective review of solitary epidermolytic acanthomas was performed at the Ackerman Academy of Dermatopathology, NY, over a 2 year period. The clinical and histopathological features of solitary epidermolytic acanthomas were described in 64 biopsies from 60 individuals. In situ hybridization for human papillomavirus (HPV) was performed on all genital lesions.
The incidence of epidermolytic acanthomas in our series was higher than previously reported (27.8/100,000). Genital location was the most common and the incidence of genital lesions was 8/100,000. In situ hybridization showed no evidence of genital HPV types within the lesions. The histological features of solitary epidermolytic acanthoma were re-evaluated.
Genital skin was the most common location for solitary epidermolytic acanthoma, but we found no evidence to suggest a role for genital HPV types. A strong male predominance was noted, and the lesions demonstrate a wide range of clinical and pathological findings.
The concurrence of Merkel cell carcinoma (MCC) and squamous cell carcinoma (SCC) is well known, and MCC concurrent with Bowen's disease has also been documented. Herein, we describe two cases of MCC concurrent with Bowen's disease, and one case exhibited an unusual immunophenotype. An 86-year-old male (Patient 1) and an 87-year-old female (Patient 2) presented with nodules of the chest and cheek, respectively. Histopathologic study revealed Bowen's disease and a proliferation of small round cells in the dermis and/or subcutis. Immunohistochemically, the round cells expressed endocrine markers. ‘Dot’ immunopositivity for cytokeratin (CK) (AE1/AE3) was observed in both patients. However, dot-like CK20 positivity was present only in the second tumor, and thyroid transcription factor-1 (TTF-1) was only positive in the first. Both cases were negative for Merkel cell polyomavirus (MCPyV). MCC concurrent with SCC usually does not involve detectable MCPyV infection, which suggests that combined MCC may develop through different tumorigenetic pathways, such as chronic ultraviolet exposure, as compared to pure MCC. Additionally, concurrent tumors may exhibit an unusual immunophenotype, such as TTF-1+/CK20(−).
]]>Primary cutaneous CD4 positive small/medium pleomorphic T-cell lymphoma (SMPTCL) represents a provisional subtype of primary cutaneous T-cell lymphoma with indolent clinical course. A few aggressive fatal cases with increased proliferation rate and few infiltrating CD8 positive T-cells have been reported. We describe a case of SMPTCL with an increased proliferation rate, admixed CD30-positive large lymphoid cells, and few infiltrating CD8 positive T-cells. The lymphoma cells were positive for CD3, CD4, CD2 and CD5, and negative for CD8. A subset of the lymphoma cells was positive for follicular helper T-cell markers bcl-6 and PD-1. There were approximately 20% CD30-positive large lymphoid cells, and Ki-67 showed a moderately high proliferation rate (∼40%), mostly in the large lymphoid cells. CD8 infiltrating T-cells were few (<5%). The patient had an indolent disease with complete response to radiation therapy. To the best of our knowledge, this is the first reported case of SMPTCL with an increased proliferation rate and large CD30+ cells that followed an indolent clinical course.
]]>Muir-Torre syndrome represents a rare autosomal dominant familial cancer predisposition disorder defined by the occurrence of cutaneous sebaceous tumors and an internal malignancy, most commonly gastrointestinal carcinoma. Most examples of hereditary non-polyposis cancer syndrome (Lynch syndrome), including the Muir-Torre syndrome, are associated with microsatellite instability (MSI) and germline mutations in mismatch repair genes—most commonly MLH1 or MSH2. We present a 58-year-old man with Muir-Torre syndrome and a large retroperitoneal mass (14.3 cm in greatest dimension) encompassing the left adrenal gland. Sections showed a cellular malignant tumor composed of spindle cells with a high mitotic index and lacking morphologic evidence of adipocytic differentiation. It was weakly reactive for smooth muscle actin (SMA) and negative for desmin, CD117, CD31, CD34, S100 protein and pan-cytokeratin. Further immunohistochemical analysis revealed intact expression of MLH1 but loss of MSH2 in tumor nuclei. Compared to non-neoplastic tissue, the tumor showed MSI in five of seven dinucleotide markers. Fluorescence in situ hybridization (FISH) failed to reveal 12q15 amplification, effectively excluding dedifferentiated liposarcoma as a diagnostic consideration. This is a rare case of a patient with Muir-Torre syndrome who developed a related high-grade undifferentiated pleomorphic sarcoma as the associated internal malignancy.
]]>Breast carcinoma remains one of the most common sources of skin metastases in women. Cutaneous breast carcinoma metastases have variable clinical and histopathologic presentations that can make diagnosis challenging. We report a unique case of metastatic breast carcinoma with prominent clear cell features, thus mimicking a xanthomatous process. Dermatopathologists should be aware of this entity given its resemblance to other clear cell infiltrates and neoplasms.
]]>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.
]]>Pleomorphic liposarcoma (PLPS) is a rare, high-grade sarcoma defined by the presence of pleomorphic lipoblasts. Constituting 5% of all liposarcomas, PLPS usually arises in deep soft tissues of the extremities, with rare occurrences in the dermis and subcutis. We describe a unique case of an 85-year-old Caucasian gentleman with a 1 year history of a pedunculated, pink, non-tender papule on the dorsum of his left arm, measuring 1.0 cm in maximum dimension. Biopsy revealed a dermal collection of atypical epithelioid and spindle cells superimposed on a sclerotic background, resembling a pleomorphic fibroma on low power. On high power, a central focus of discrete adipocytic differentiation with pleomorphic lipoblasts was present. Tumor cells were positive for S-100 and negative for desmin, actin, CD68, keratin, MART-1 and CD34. Clinicopathologic findings were consistent with PLPS and the diagnosis was made. PLPS is rarely localized to the dermis and one with low power features resembling a pleomorphic fibroma has not been previously described in the literature.
]]>Recent reports of 10 patients have proposed a papular variant of mycosis fungoides (MF), characterized by the appearance of papules in the absence of patches and the presence of histopathologic features of classic patch/plaque stage MF. Given the overlapping clinical and pathologic features between this proposed entity and lymphomatoid papulosis (LyP)-type B, however, the optimal classification for such cases remains somewhat unclear. Herein, two patients are described who presented to the dermatology clinic with persistent erythematous papular eruptions on the trunk, upper and lower extremities and histopathology compatible with MF. Of note, these patients represent the oldest reported cases of papular MF, and one patient had documented peripheral blood involvement by atypical CD4+ cells. The clinicopathologic characteristics of this purported entity suggest that it may occupy the intersection between MF and CD30+ lymphoproliferative disorders. These two cases expand the clinicopathologic spectrum of papular MF to include older individuals, and further emphasize the importance of recognition of this morphology as a possible MF manifestation. Furthermore, consideration of our cases in conjunction with the previously documented 10 other patients in the literature, offers potential insight into the relationship between MF and CD30+ lymphoproliferative disorders.
]]>Pseudolymphomatous cutaneous angiosarcoma represents a rare, relatively new variant of cutaneous angiosarcoma exhibiting a prominent inflammatory lymphoid infiltrate that can mask the underlying vascular malignant proliferation and mimic a lymphomatous or pseudolymphomatous process. We describe the clinicopathologic characteristics of two new cases of pseudolymphomatous cutaneous angiosarcoma whose originality lies in the unusual setting from which they have arisen. In fact, the first case was an exceedingly lymphocyte-rich recurrence of a typical epithelioid cutaneous angiosarcoma whose primary lesion that was almost devoid of inflammatory infiltrate underwent surgical excision and radiotherapy while the second one was an unexpected histopathological finding associated with a basal cell carcinoma. Immunohistochemically, most of the lymphocytes expressed immunoreactivity for T-cell markers, while the neoplastic endothelial lymphatic cells expressed CD31 and CD34. D2-40 immunoreactivity was observed lining some channels and some neoplastic cells. In the first case a possible relationship between radiotherapy and the pseudolymphomatous reactive pattern is discussed while the second case has been considered as a rare example of collision tumor.
]]>Skin infiltration by chronic lymphocytic leukemia (CLL) is very rare and almost all reported cases occur in advanced stage. We report a patient with no relevant past medical history who presented with cutaneous erythematous plaques. A punch biopsy showed typical CLL morphologic and immunophenotypic features. Subsequent studies revealed a normal lymphocyte count in peripheral blood, and there was no evidence of lymphadenopathy or organomegaly. Flow cytometry demonstrated a clonal B-cell population both in the bone marrow and peripheral blood (1.60 × 109/l) with a CLL phenotype, but it did not fulfill required criteria for CLL diagnosis. Without cutaneous involvement, this case should be classified as monoclonal B-cell lymphocytosis.
]]>Generalized eruptive histiocytosis, described in 1963 by Winklemann and Muller, is a reactive, self-healing form of non-Langerhans histiocytosis. Rare cases of atypical generalized eruptive histiocytosis have been reported in patients with hematopoietic malignancy, but the biological relationship between the two disorders is not known. We report an 84-year-old man with chronic myelomonocytic leukemia who presented with coalescing erythematous papules and plaques on the posterior neck, ear and lower lip, followed by development of blast crisis. Skin biopsy revealed a thick band-like dermal infiltrate of cells that exhibited morphologic features of macrophages or histiocytes and prominent elastolytic phagocytosis. These cells demonstrated a mature immunophenotype, expressing CD14 and CD68, with partial expression of CD13 but not CD1a, CD43, CD56, CD123, Langerin, or S-100 protein. Karyotype and fluorescence in situ hybridization analyses showed loss of the Y chromosome in bone marrow and skin specimens, providing evidence of a clonal relationship between the cutaneous eruption and the underlying chronic myelomonocytic leukemia. The presence of the same clone in skin and bone marrow specimens from our patient supports the possibility that atypical generalized eruptive histiocytosis is a marker for underlying hematopoietic malignancy. Discovery of additional cases may shed further light on the pathogenesis of this rare entity.
]]>Follicular mucinosis represents a term for a histopathologic reaction pattern in follicular epithelium. It is a characteristic of alopecia mucinosa. However, it may also occur in a variety of unrelated conditions. Epidermal nevi are considered to be hamartomatous disorders and they can show a predominant component of non-organoid (keratinocytes) and/or organoid nevi. All the cases of epidermal nevi described with mucin deposits until now are reported as mucinous nevus or mucinous eccrine nevus; in the first type of disorder, diffuse mucin deposition is only seen in the papillary dermis, and in the second type, the mucin is found around the proliferation of eccrine structures. We believe this is the first reported case of epidermal nevus along Blaschko's lines exhibiting typical microscopic findings of mucinosis exclusively distributed inside the follicular epithelia.
EMLA® (eutectic mixture of local anesthetics, 2.5% each of lidocaine and prilocaine in an oil and water emulsion) is used as a topical anesthetic. We report three cases of EMLA®-induced histopathologic changes on the vulvar epithelium. While there are some similar histopathologic features to those reported in extragenital skin, we describe additional findings on vulvar epithelium, which, to our knowledge, have not been reported previously. The patients presented with clinical signs suggestive of lichen sclerosus or erosive lichen planus (LP), but were all confirmed histopathologically as LP. The biopsy was taken after 15 min of EMLA® application and intradermal injection of 1% lidocaine. Blistering prior to intradermal lidocaine and the biopsy procedure was observed in two patients. The histopathologic changes observed in the epithelium included pallor of the upper epidermis, mild spongiosis, intraepidermal subcorneal and suprabasal acantholysis, congestion of the papillary dermal capillaries and extravasated erythrocytes. Basophilic granules were present, but rare, while the necrosis with multifocal clefting was more marked than in extragenital skin. It is important to be aware of these changes occurring on genital mucosa; these may occur in the absence of clinical signs and may obscure the primary underlying pathology, thus representing a diagnostic pitfall.
]]>The occurrence of a tumor at the colostomy site after abdominoperineal resection for rectal carcinoma is rare and it may be related to a previously resected carcinoma or another primary tumor. We report a 61-year-old man who developed an ulcerated skin nodule at her colostomy site 6 years after resection of a rectal adenocarcinoma. Histopathologically, the skin nodule was composed of atypical large and pleomorphic cells with high mitotic rate and they were arranged in nests and within lymphatic channels in the dermis. The neoplastic cells were immunoreactive for cytokeratin (CK) AE1/3, CK7, CK34ßE12, epithelial membrane antigen and vimentin while detection of human papillomavirus and Epstein–Barr virus DNA was negative. A diagnosis of basaloid large cell carcinoma of pulmonary origin was suggested and it was confirmed by computed tomography-guided fine needle aspiration of a right subpleural mass. A metastatic tumor at the colostomy site is an exceptional finding and may be the first manifestation of lung cancer, especially if it consist of pleomorphic large cells with high mitotic rate and basaloid immunophenotype.
]]>Cutaneous gamma-delta T-cell lymphoma (γδTCL) is a rare malignancy that typically displays an aggressive clinical course. We present an unusual case of a 57-year-old woman with a 3-year history of lower extremity nodules. Histopathologic, immunophenotypic and molecular genetic studies revealed a clonal, predominantly pannicular gamma-delta T-cell infiltrate, leading to a diagnosis of cutaneous γδTCL. The clinical course was characterized by rapid improvement within months of starting systemic corticosteroids, with relapse in ulcerations but no new lesions more than 3 years after onset of disease. Our case and seven previously reported patients with indolent and relatively localized cutaneous γδTCL provide evidence that not all cases of this entity carry a poor prognosis. This indolent subset adds complexity to treatment of cutaneous γδTCL.
]]>Terminology regarding classification of benign lesions with prominent eccrine differentiation can be confusing as these lesions can have overlapping clinical and histologic characteristics. In this report, we examine a case and review of the literature to suggest that these entities may be better classified as a spectrum of benign lesions with overlapping features rather than distinct entities. We describe a case of an acantholytic dyskeratotic epidermal nevus with eccrine differentiation on the back of a 2-year-old patient. We then discuss the classic clinical and histologic presentations of eccrine nevi and epidermal nevi with eccrine differentiation as they relate to each other and to our case.
]]>Background: We planned this study to analyze probable associations between p53, cyclinD1, Ki67 and histopathological features in basal cell carcinomas (BCC).
Methods: Histological differentiation types, histological growth patterns and tissue responses were analyzed in 50 cases of BCC. In immunohistochemical analysis, p53, cyclinD1 and Ki67 antibodies were investigated. P53 expression was evaluated based on a cut-off value of 25% positivity. CyclinD1 expression was graded from 0 to 3+ according to the percentage of positive nuclear staining. The percentage of positively staining cells for Ki67 was recorded.
Results: The following significant correlations were detected. Solid infiltrative type differentiation was related to the infiltrative histological growth pattern. The rates of p53 positivity and severe fibrosis in the groups of mixed and infiltrative growth patterns were higher than others. Besides, p53-positive cases showed more severe fibrosis and had a higher mean value for Ki67 index. Epidermal p53 and cyclinD1 clones in normal epidermal areas adjacent to tumors were noticed in 42% and 52% of the cases, respectively.
Conclusions: P53 expression seems to be related to Ki67 index and some histopathological features of BCC, such as infiltrative histological growth pattern and probably fibrosis.
The purpose of this study was to explore the immunohistochemical and mutational status of the tyrosine kinases KIT and platelet derived growth receptor-alpha (PDGFRA) in Merkel cell carcinoma (MCC). Specifically, we examined the mutated exons in gastrointestinal stromal cell tumors that may confer a treatment response to imatinib mesylate.
We evaluated KIT and PDGFRA immunostaining in 23 examples of MCC utilizing laser capture microdissection to obtain pure samples of tumor genomic DNA from 18 of 23 examples of MCC. PCR amplification and sequencing of KIT exons 9, 11, 13 and 17, and PDGFRA exons 10, 12, 14 and 18 for mutations was performed.
Fifteen of 23 tumors (65%) demonstrated CD117 expression and 22 of 23 tumors (95%) demonstrated PDGFRA expression. A single heterozygous KIT exon 11 base change resulting in an E583K mutation was discovered in 12 of 18 (66%) examples of MCC. In addition, a single nucleotide polymorphism was detected in eight of 18 tumors (44%) in exon 18 of PDGFRA (codon 824; GTC > GTT).
We discovered a novel somatic KIT exon 11 E583K mutation in 66% of tumors. This mutation has been previously described in a human with piebaldism and appears to represent an inactivating mutation. Therefore, despite expression of CD117 and PDGFRA, the absence of activating mutations in these tyrosine kinases makes KIT and PDGFRA unlikely candidates of MCC oncogenesis.
Sebaceous carcinoma represents a rare and potentially fatal adnexal malignancy. Poorly-differentiated sebaceous carcinoma consisting of infiltrative basaloid tumor cells with inapparent lipid vesicles can mimic basal cell carcinoma (BCC). Conversely, other epithelial tumors can exhibit clear cell histopathology and mimic sebaceous carcinoma. At the present time, immunohistochemical markers unique for sebaceous carcinoma are limited.
We evaluated the expression of three lipid synthesis/processing protein markers alpha/beta hydrolase domain-containing protein 5 (ABHD5), progesterone receptor membrane component-1 (PGRMC1) and squalene synthase (SQS) in sebaceous carcinoma and investigated their utility in differentiating sebaceous tumors from BCC with clear cell features. Immunohistochemistry was performed on 23 sebaceous carcinomas, 14 sebaceomas and 14 BCCs with clear cell features.
In sebaceous carcinomas, ABHD5 showed dispersed, cytoplasmic punctate and/or vesicular staining (n = 19/23, 83%), while PGRMC1 and SQS each showed vesicular and membranous staining in tumor cells (n = 22/23, 96%). In all sebaceomas, these markers highlighted tightly clustered lipid vesicles in sebocytes. All BCCs with clear cell features were negative for these three markers.
ABHD5, PGRMC1 and SQS are novel markers for sebaceous carcinoma and can reliably distinguish sebaceous neoplasms from non-sebaceous tumors, specifically BCC with clear cell features.
Myxofibrosarcoma (MFS) arises most commonly in the proximal extremities of the elderly, where it may involve subcutaneous and dermal tissues and masquerade as benign entities in limited biopsy samples. We encountered such a case, in which positivity for CD34 and morphologic features were initially wrongly interpreted as a ‘low-fat/fat-free’ spindle cell/pleomorphic lipoma. Case series have not assessed prevalence of CD34 reactivity among cutaneous examples of MFS.
We performed a systematic review of our institution's experience, selecting from among unequivocal MFS resection specimens those superficial cases in which a limited biopsy sample might prove difficult to interpret. These cases were immunostained for CD34 and tabulated for clinicopathologic characteristics.
After review of all MFS diagnoses over 5 years (n = 56), we identified a study group of superficial MFS for comparison to the index case (total n = 8). Of these, the index and three additional cases (4 of 8, 50%; 2 low, 2 high grade) demonstrated positive staining for CD34, with diffuse staining of spindled cells including cellular processes. Four additional cases showed no or equivocal/rare staining.
CD34 positivity should be recognized as prevalent among such cases and should not be inappropriately construed as inveighing against a diagnosis of MFS in favor of benign entities.
Cytokeratin 7 (CK7) and Cam 5.2 are often used to differentiate extramammary Paget's disease (EPD) from squamous cell carcinoma (SCC) in situ because they are generally considered to be expressed in the former but not in the latter. However, we have encountered CK7+ and Cam 5.2+ SCCs.
We evaluated CK7, Cam 5.2 and Ber-Ep4 expression in SCC and EPD.
We found significant CK7 and Cam 5.2 positivity in SCCs, particularly in those with a pagetoid pattern. Only one case expressed Ber-Ep4.
We conclude that CK7 and Cam 5.2 expression may occur in SCC. A panel including Ber-Ep4 is advisable for immunohistochemical differentiation of EPD from SCC.
The contribution of the E-cadherin transcriptional repressors Snail and Slug to invasion and metastasis has strengthened the evidence for the importance of epithelial-mesenchymal transition (EMT) in carcinoma progression. However, to the best of our knowledge, no study has described the immunohistochemical staining of the EMT-related proteins Snail/Slug in skin tumors and the correlation between Snail/Slug and tumor suppressor p53/p63.
We performed immunohistological staining of Snail, Slug, E-cadherin, p53 and p63 in 20 archived specimens each of seborrheic keratosis (SK), actinic keratosis (AK) and squamous cell carcinoma in situ (SCCIS), and 53 specimens of cutaneous squamous cell carcinomas (SCC). Fifteen normal skin (NS) specimens served as controls.
Significant negative correlations were observed between Snail and E-cadherin expression and between Slug and E-cadherin expression (Snail: R2 = 0.5432, p < 0.01; Slug: R2 = 0.4666, p < 0.01).
The staining intensities of Snail and Slug are associated with decreased E-cadherin staining in SCC and this may promote EMT. However, the staining intensities of p53 and p63 are not significantly correlated with the loss of E-cadherin.
Cutaneous lymphatic malformations represent a group of heterogeneous diseases caused by developmental defects of lymphatic system.
The purpose of this study was to report the clinical, histopathological and immunohistochemical features of a distinctive lymphatic malformation.
Twelve patients with similar clinical and histopathological features were included in this study. Immunohistochemical staining of CD31, D2-40, Prox1 and Wilms tumor 1 (WT-1) were performed on all lesions.
All cases were either congenital lesions or developed during the first 2 years of life. All presented as red to brown papules or nodules on acral sites. Histopathologically, the lesions consisted of a dermal proliferation of flat or slit-like vessels lined with a single layer of endothelial cells. Hemosiderin or extravascular red blood cells were present in all cases. The constituent vessels expressed CD31, D2-40 and Prox1 and lacked expression of WT-1.
On the basis of the clinical, histopathological and immunohistochemical findings, our cases represent a unique type of lymphatic malformation that we believe is distinct from previously reported vascular proliferations. We propose the name of acral hemosideric lymphatic malformation for this entity.
The presence of non-cutaneous vascular lesions in the syndrome of multiple enchondromas and subcutaneous hemangiomas, also named Maffucci syndrome, is exceedingly rare. Until now, non-cutaneous vascular lesions have been described in nine patients, while only three cases were present in the oral cavity; they were found in the tongue in two patients and in the lower lip in one patient. Herein, we report the second case of vascular lesions localized in the mucosa of lower lip in a patient with Maffucci syndrome. Histopathologic examination showed spindle cell hemangioma.
]]>Vemurafenib is an inhibitor of BRAF and is used to treat patients with metastatic melanoma who carry a V600E BRAF mutation. Recently, four patients have been described in the literature who developed a neutrophilic panniculitis following treatment with a BRAF inhibitor. We present an additional case and review the clinical findings of the cases reported to date.
]]>Primary cutaneous neoplasms of histiocytes and dendritic cells are rare. Langerhans cells are a subset of antigen-presenting dendritic cells. Neoplasms of Langerhans cells are classified into cytologically benign Langerhans cell histiocytosis and cytologically malignant Langerhans cell sarcoma. Langerhans cell sarcoma is a rare entity characterized by multiorgan involvement and an aggressive clinical course. To date, only 30 cases of Langerhans cell sarcoma, including the present case, have been reported. We report a new case of Langerhans cell sarcoma that presented with multifocal cutaneous involvement. Diagnosis was done based on histopathological, immunohistochemical evaluation, as well as ultrastructural analysis identifying the presence of Birbeck granules. Our case represents a new case of this extremely rare, overtly aggressive neoplasm of Langerhans cells. Within 2 years of diagnosis, the patient developed metastatic disease and consequently died. Early recognition is important because of the tendency of Langerhans cell sarcoma to recur and metastasize. Therefore, ancillary techniques such as immunohistochemical and ultrastructural studies to confirm the diagnosis are very advantageous.
]]>We report a case of a 48-year-old Malay female who presented with multiple tumors arising from a large nevus sebaceus on her right parieto-temporal scalp. Histologically, the tumors corresponded to a sebaceoma with carcinomatous change, a poroma and a trichoblastoma. Immunohistochemical staining of the sebaceous tumor with p53 showed strong within the areas of carcinomatous change, while there was negative to weak staining within the sebaceoma-like areas. A discussion on the potential secondary neoplasms from a nevus sebaceus ensues, with a review of this literature on multiple tumors from a nevus sebaceus.
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