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Internal Malignancies (internal + malignancy)
Selected AbstractsCancer-associated genodermatoses: a personal historyEXPERIMENTAL DERMATOLOGY, Issue 9 2006Walter H. C. Burgdorf Abstract:, Cancer-associated genodermatoses are a group of genetic disorders inherited in an autosomal-dominant fashion in which unique cutaneous findings are a reliable marker for the risk of developing internal malignancies. The historical, clinical and dermatopathological aspects of basal cell nevus syndrome, Muir,Torre syndrome, Cowden syndrome, Carney complex and Birt,Hogg,Dubé syndrome are reviewed in a personal and informal fashion. The latest advances in the molecular genetics of the disorders are also summarized. [source] Two Japanese cases of lichen planus pigmentosus-inversusINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 7 2007Aki Kashima MD Case 1 was a 51-year-old Japanese woman. She presented with an asymptomatic brown macule located on the right axilla of 2 months' duration. The smooth macule was 2 cm in diameter with a sharp demarcation (Fig. 1A). Figure 1. Photographs of skin lesions in two patients. (A) Case 1. Well-circumscribed brown macule without an active red border in the central portion of the right axilla. (B) Case 2. Symmetric distribution of brown macules without an active red border in the popliteal fossae Case 2 was a 62-year-old Japanese man. He presented with asymptomatic, symmetric, gray,brown macules located on the groin, axillae, and popliteal region of 6 months' duration. The smooth macules were several millimeters to centimeters in diameter and sharply demarcated (Fig. 1B). Oral or nail lesions, previous inflammatory processes in affected areas, and internal malignancies were absent. A causal relationship with drugs, recent sun exposure, or trauma could not be identified. Findings for work-up, including blood cell count, fasting blood sugar levels, liver function, serum electrolyte levels, serum electrophoresis, urinalysis, antinuclear antibodies, and serological examinations for human hepatitis viruses and syphilis, were within normal limits or negative. The lesions gradually disappeared without medication within 6 months. Biopsy specimens showed a lymphocytic infiltrate with basal vacuolar changes and prominent melanin incontinence in the upper dermis (Fig. 2A). The band-like lymphocytic infiltrate was moderate in Case 1 and mild in Case 2. Immunohistochemistry showed infiltrative CD8+ T lymphocytes with keratinocytic damage, indicating cytotoxic injury of the keratinocytes (Fig. 2B). Both the epidermis and the upper dermis contained CD1a+ cells (Fig. 2C). The keratinocytes focally and weakly expressed HLA-DR (Fig. 2D). These findings were identical in samples from both patients. Figure 2. Light and immunohistochemical microphotographs. (A) Mild, band-like, lymphocytic infiltrate with basal vacuolar change and prominent melanin incontinence in the upper dermis with apoptosis or necrosis of keratinocytes. (B) Epidermal infiltrate of CD8+ T lymphocytes with keratinocytic damage. (C) CD1a+ cells in the upper dermis. (D) Keratinocytes focally and weakly express HLA-DR (original magnifications: A, ×200; B,D, ×400) [source] Cutaneous sebaceous neoplasms as markers of Muir-Torre syndrome: a diagnostic algorithmJOURNAL OF CUTANEOUS PATHOLOGY, Issue 6 2009Ossama Abbas Sebaceous gland neoplasms such as adenoma, epithelioma, and carcinoma are uncommon cutaneous tumors. Although sporadic, their occurrence is clinically significant because of their association with Muir-Torre syndrome (MTS). MTS is a rare autosomal dominant genodermatosis characterized by the occurrence of sebaceous gland neoplasms and/or keratoacanthomas associated with visceral malignancies that include gastrointestinal and genitourinary cancers. MTS is usually the result of germline mutation in one or more of the DNA mismatch repair (MMR) genes. MMR genes commonly implicated include MSH -2 and MLH -1 and, more recently, MSH -6. Recent evidence suggests that immunohistochemistry is very sensitive and effective in detecting these defects in cutaneous tumors in MTS. In addition, the genetic instability of cutaneous and visceral tumors in MTS caused by the defects in MMR genes can also be detected, using polymerase chain reaction (PCR)-based techniques, for microsatellite instability (MSI). Given that some sebaceous neoplasms represent cutaneous markers of MTS, what should we as dermatopathologists be advocating? Should we be looking for absence/loss of MMRs in all sebaceous neoplasms? When should we recommend assaying for MSI? This review attempts to address all of these issues with a view to streamlining the work-up of a patient presenting for the first time with a sebaceous neoplasm and no prior personal or family history of internal malignancies. [source] Bullous pemphigoid in Liguria: A 2-year surveyJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 4 2001E Cozzani Abstract Background The epidemiology of bullous pemphigoid (BP) is not clear because of the heterogeneity of the disease, and its possible association with internal malignancies has been under debate for many years. We report the findings of a 2-year study on incident BP cases in the Liguria region of Italy. Subjects and methods Thirty-two patients with BP were collected over the 2-year period. Diagnosis was made based on clinical findings and confirmed by histology, direct immunofluorescence (DIF) and indirect immunofluorescence (IIF) with salt-split skin and monkey oesophagus, and immunoblotting (IB). All patients were thoroughly investigated for possible malignancies and all were followed up for 6 months to monitor the response to treatment. Results DIF showed linear deposits at the dermoepidermal junction in all but one patient. IIF gave positive findings for 15 sera tested with monkey oesophagus and 20 tested with salt-split skin. IB gave positive findings in 19 cases. There was a malignancy in six cases, but no clinical or immunological features that could be considered to predict this occurrence. Conclusions The findings of this study are in accordance with most of the data found in the literature, including the fact that IgG serum levels did not predict the course of the disease. Contrary to previous indications, IgE levels were not indicative of disease course either. Mucosal lesions, erythema multiform-like lesions, negative IIF findings and antibodies to AgPB2 were not a prediction for the development of malignancy. [source] Bullous pemphigoid as a dermadrome associated with spindle cell carcinoma of the gallbladderTHE JOURNAL OF DERMATOLOGY, Issue 3 2010Ayano UMEKOJI Abstract Skin disorders that appear in association with internal malignancies are called dermadromes. Bullous pemphigoid (BP), which is a major autoimmune disease of the skin, is considered to be a dermadrome, although there have been conflicting reports. We report a case of BP that preceded the diagnosis of an internal malignancy. Although we could not detect any malignancies on chest, abdominal or pelvic computer tomography on the first hospital admission, intensive screening on the third admission revealed a gallbladder malignancy. Laparoscopic cholecystectomy was performed. Histopathology showed a spindle cell carcinoma of the gallbladder. To the best of our knowledge, this is the first report of a spindle cell carcinoma of the gallbladder in a patient with BP. [source] Identification of Muir,Torre syndrome among patients with sebaceous tumors and keratoacanthomasCANCER, Issue 5 2005Role of clinical features, immunohistochemistry, microsatellite instability Abstract BACKGROUND The Muir,Torre syndrome (MTS) is an autosomal-dominant genodermatosis characterized by the presence of sebaceous gland tumors, with or without keratoacanthomas, associated with visceral malignancies. A subset of patients with MTS is considered a variant of the hereditary nonpolyposis colorectal carcinoma, which is caused by mutations in mismatch-repair genes. The objective of the current study was to evaluate whether a combined clinical, immunohistochemical, and biomolecular approach could be useful for the identification of Muir,Torre syndrome among patients with a diagnosis of sebaceous tumors and keratoacanthomas. METHODS The authors collected sebaceous skin lesions and keratoacanthomas recorded in the files of the Pathology Department of the University of Modena during the period 1986,2000. Through interviews and examination of clinical charts, family trees were drawn for 120 patients who were affected by these skin lesions. RESULTS Seven patients also were affected by gastrointestinal tumors, thus meeting the clinical criteria for the diagnosis of MTS. In the MTS families, a wide phenotypic variability was evident, both in the spectrum of visceral tumors and in the type of skin lesions. Microsatellite instability was found in five MTS patients: These patients showed concordance with immunohistochemical analysis; moreover, a constitutional mutation in the MSH2 gene was found in 1 patient. Lack of expression of MSH2/MSH6 or MLH1 proteins was evident in the skin lesions and in the associated internal malignancies of 3 patients and 2 patients with MTS, respectively. CONCLUSIONS The clinical, biomolecular, and immunohistochemical characterization of sebaceous skin lesions and keratoacanthomas may be used as screening for the identification of families at risk of MTS, a disease that is difficult to recognize and diagnose. Cancer 2005. © 2005 American Cancer Society. [source] Extramammary Paget's disease of the groin with underlying carcinoma and fatal outcomeCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 5 2008J. C. Pascual Summary Extramammary Paget's disease (EMPD) is considered to be an intraepithelial adenocarcinoma. Typically involved anatomical sites are the vulvar, perianal, perineal, scrotal and penile regions. Clinically, the lesions present as well-defined, moist, erythematous plaques usually accompanied by pruritus. An unusual feature of EMPD is its association with cutaneous, adnexal-structure adenocarcinomas and its association with internal malignancies. Histopathological examination shows epidermal acanthosis and elongated rete ridges. Paget's cells are large intraepidermal cells with a large nucleous and abundant pale cytoplasm. Recent studies of perianal and vulvar EMPD have described distinct immunohistochemical subtypes termed cutaneous and endodermal. Cutaneous EMPD is characteristically positive for cytokeratin (CK)7, negative for CK20, and positive for gross cystic disease fluid protein (GCDFP)15+, whereas endodermal EMPD shows a CK7+ CK20+ GCDFP15, phenotype. Surgery remains the treatment of choice, with either wide surgical excision or Mohs' micrographic surgery. We present a case of EMPD with an underlying carcinoma, which combined immunohistochemical findings suggestive of the cutaneous subtype (positive for CK7, GCDFP15, mucin (MUC)1, human epidermal growth factor receptor (HER)2/neu positive) and the endodermal subtype, frequently associated with internal malignancy (CK20, MUC2, CDX-2 positve); however, our patient had no associated internal malignancy. [source] Subcorneal pustular dermatosis: 50 years onCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 3 2008S. Cheng Summary We review the key developments in our understanding of subcorneal pustular dermatosis (SCPD, also known as Sneddon,Wilkinson disease) over the past 50 years. SCPD is a rare, chronic, sterile pustular eruption that was first described by Sneddon and Wilkinson in 1956. The primary lesions are pea-sized pustules classically described as half-pustular, half-clear flaccid fluid blisters. Histologically the salient feature is a subcorneal accumulation of neutrophils, suggesting the presence of chemoattractants such as tumour necrosis factor (TNF), in the uppermost epidermis. However, to date its exact pathophysiology is unknown. Cases in association with pyoderma gangrenosum, benign monoclonal IgA gammopathy and multiple myeloma are well documented. There are anecdotal reports of SCPD associated with other internal malignancies such as chronic lymphocytic leukaemia, thymoma, apudoma and epidermoid carcinoma of the lung. The treatment of choice is dapsone. Therapeutic alternatives include retinoids, phototreatment with psoralen ultraviolet (UV) A, broadband or narrow band UVB and corticosteroids. Anecdotal uses of tacalcitol, ketoconazole, azithromycin, tetracycline, minocycline, vitamin E, ciclosporin, colchicine, mizoribine, mebhydrolin, infliximab and adalimumab with mycophenolate mofetil have all been reported. [source] Retrospective analysis of the occurrence of internal malignancy in patients treated with PUVA between 1986 and 1999 in South WarwickshireCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 2 2004J. E. Gach Summary Concerns were raised in our department when four of our patients receiving PUVA treatment developed internal malignancy. We reviewed the medical and phototherapy case notes of patients who received either systemic or bath PUVA therapy in our department between 1986 and 1999. Among the 197 patients for whom we were able to trace the hospital records we identified five patients with internal malignancies. Over the same period (1986,1999) we calculated, using the Kaplan,Meier nonparametric estimator, that 4.6 cases of internal malignancy would have been anticipated in our study population. Therefore PUVA therapy did not appear to be a risk factor for internal malignancy. [source] Skin findings in internal malignant diseasesINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 10 2007Arzu K Purpose A skin finding may be an indicator of internal malignant diseases. In this report, we investigated the skin findings of the cases who have had internal malignancy within the last 1 month. Patients and methods Seven hundred cases who were diagnosed as internal malignancy and who did not have any treatment for the malignancy were enrolled in our study between February 2002 and September 2003. A form was completed for all of the cases, including name, surname, and the carcinoma type. All cases were examined in detail, and the observed skin findings or dermatosis was recorded. Results The most frequent skin findings among the cases were tinea pedis/onychomycosis, followed by xerosis and pruritus. The skin findings in terms of frequency were determined mostly in hematological malignancies (68.96%). Conclusion We would like to emphasize that the skin is an indicator of the functions of internal organs and their disorders. [source] Cutaneous metastasis: a clinical, pathological, and immunohistochemical appraisalJOURNAL OF CUTANEOUS PATHOLOGY, Issue 6 2004Sadia Saeed Background:, Cutaneous tumor metastasis may be the first manifestation of cancer, but more often is a harbinger of advanced disease that portends an ominous prognosis. All skin accessions over the past 10 years from a large Veterans Administration (VA) hospital were reviewed. Methods:, Archived histories, glass slides, and the immunohistochemical battery (IHC), were assessed to determine diagnostic accuracy. Results:, Of the 100,453 cases reviewed, there were a total of 77 cases (75 males and 2 females) of cutaneous metastasis from the lungs (28.6%), metastatic melanoma (18.2%), gastrointestinal tract (14.2%), genitourinary tract (10.4%), head and neck (9.1%), hematologic (5.2%), breast (5.2%), and miscellaneous (<2%). Metastasis represented the first indication of an internal malignancy in 7.8% of cases. The cutaneous sites of involvement included the head and neck (28%), the trunk (40%), the extremities (18%), and multiple sites (14%). The age range was 38,83 years, with a mean of 62 years. The average time interval between diagnosis of internal malignancy and cutaneous presentation was 33 months (range: <1 month,22 years), and the average survival following diagnosis was 7.5 months (range: <1 month,8 years). In a cohort of subjects, a truncated immunohistochemical battery consisting of CK-7, CK-20, and S-100 was consistent with the expected staining pattern of the primary source of cutaneous metastasis in 83.33% of the patients. Conclusions:, Excluding the potential for age and gender bias in this study conducted in a VA setting, cutaneous metastases represent an uncommon, deadly, and late-developing occurrence in many patients. Compared with previous studies, lung carcinoma remains the most common of the cutaneous metastases, with a relative rise in the incidence of metastatic melanoma. The immunohistochemical battery of CK-7, CK-20, and S-100 is a helpful adjunct in narrowing the differential diagnosis of the primary site of a large proportion of cutaneous metastases, particularly tumors with an epithelioid appearance such as carcinomas and melanomas. [source] Florid cutaneous and mucosal papillomatosis with acanthosis nigricans revealing a primary lung cancerJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 3 2000Ugo Bottoni Abstract This is the report of an 80-year-old patient with diffuse brownish hyperpigmentation and velvety thickening of the skin with onset 1 year before. Warty lesions on his limbs were present as well as papillomatous and verrucous lesions on his lips, mouth and eyelid conjunctivae with hyperkeratosis of the nipples. Biopsies, performed at different sites, showed histological pictures consistent with a diagnosis of acanthosis nigricans (AN) with florid cutaneous and mucosal papillomatosis. This type of AN is frequently associated with internal malignancy. In our patient serum levels of tissue polypeptide antigen, carcinoembryonic antigen, cytokeratin fragment and squamous cell carcinoma antigen were high and chest computed tomography scan indicated a large tumour infiltrating the right lung and extending to the mediastinum. Cytological examination of bronchial drainage revealed the presence of neoplastic cells, non-small cell type carcinoma. The most frequent cancer associated with malignant AN is gastric adenocarcinoma. Lung tumour has rarely been reported with AN. Malignant AN is sometimes associated with other cutaneous and mucosal warty lesions, as in our patient. These various skin and mucosal lesions are the expression of a systemic epithelial disorder and may help clinicians to suspect a malignant form of AN. [source] Bullous pemphigoid as a dermadrome associated with spindle cell carcinoma of the gallbladderTHE JOURNAL OF DERMATOLOGY, Issue 3 2010Ayano UMEKOJI Abstract Skin disorders that appear in association with internal malignancies are called dermadromes. Bullous pemphigoid (BP), which is a major autoimmune disease of the skin, is considered to be a dermadrome, although there have been conflicting reports. We report a case of BP that preceded the diagnosis of an internal malignancy. Although we could not detect any malignancies on chest, abdominal or pelvic computer tomography on the first hospital admission, intensive screening on the third admission revealed a gallbladder malignancy. Laparoscopic cholecystectomy was performed. Histopathology showed a spindle cell carcinoma of the gallbladder. To the best of our knowledge, this is the first report of a spindle cell carcinoma of the gallbladder in a patient with BP. [source] Extramammary Paget's disease of the groin with underlying carcinoma and fatal outcomeCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 5 2008J. C. Pascual Summary Extramammary Paget's disease (EMPD) is considered to be an intraepithelial adenocarcinoma. Typically involved anatomical sites are the vulvar, perianal, perineal, scrotal and penile regions. Clinically, the lesions present as well-defined, moist, erythematous plaques usually accompanied by pruritus. An unusual feature of EMPD is its association with cutaneous, adnexal-structure adenocarcinomas and its association with internal malignancies. Histopathological examination shows epidermal acanthosis and elongated rete ridges. Paget's cells are large intraepidermal cells with a large nucleous and abundant pale cytoplasm. Recent studies of perianal and vulvar EMPD have described distinct immunohistochemical subtypes termed cutaneous and endodermal. Cutaneous EMPD is characteristically positive for cytokeratin (CK)7, negative for CK20, and positive for gross cystic disease fluid protein (GCDFP)15+, whereas endodermal EMPD shows a CK7+ CK20+ GCDFP15, phenotype. Surgery remains the treatment of choice, with either wide surgical excision or Mohs' micrographic surgery. We present a case of EMPD with an underlying carcinoma, which combined immunohistochemical findings suggestive of the cutaneous subtype (positive for CK7, GCDFP15, mucin (MUC)1, human epidermal growth factor receptor (HER)2/neu positive) and the endodermal subtype, frequently associated with internal malignancy (CK20, MUC2, CDX-2 positve); however, our patient had no associated internal malignancy. [source] Retrospective analysis of the occurrence of internal malignancy in patients treated with PUVA between 1986 and 1999 in South WarwickshireCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 2 2004J. E. Gach Summary Concerns were raised in our department when four of our patients receiving PUVA treatment developed internal malignancy. We reviewed the medical and phototherapy case notes of patients who received either systemic or bath PUVA therapy in our department between 1986 and 1999. Among the 197 patients for whom we were able to trace the hospital records we identified five patients with internal malignancies. Over the same period (1986,1999) we calculated, using the Kaplan,Meier nonparametric estimator, that 4.6 cases of internal malignancy would have been anticipated in our study population. Therefore PUVA therapy did not appear to be a risk factor for internal malignancy. [source] Erythema gyratum repens and acquired ichthyosis associated with transitional cell carcinoma of the kidneyCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 6 2001M. Ameen Both erythema gyratum repens (EGR) and acquired ichthyosis are distinctive dermatoses which have strong associations with internal malignancy. EGR usually precedes the diagnosis of malignancy whereas acquired ichthyosis commonly manifests after the detection of malignancy. We report a patient who initially presented with a figurate eruption of EGR which later developed into a widespread ichthyosis with disappearance of the serpiginous rash. Further investigations revealed an underlying transitional cell carcinoma of the kidney, an association which has not previously been reported with either EGR or acquired ichthyosis. The occurrence of two paraneoplastic skin disorders in the same patient may be explained by tumour cell secretion of transforming growth factor alpha, which has been shown to be mitogenic for keratinocytes. [source] | ||||||||||