The risk of metastases from squamous cell carcinoma of the skin.

Cutaneous squamous cell carcinoma (cSCC) is the second most common nonmelanoma skin cancer, accounting for 20% of all skin cancers. The risk of the disease continues to rise annually with an estimated 50-200% increase in incidence within the last three decades. Although cSCC is one of the most common skin cancers, reported having 1 million cases per year in the United States, there is inconsistency with its reported metastatic rate. Research exploring cSCC metastasis found an overall rate of 1.2-5% but this range varies, and some dermatologists are finding cSCC's ability to spread more worrisome. This allows for clinical variation in the appropriate treatments and follow-up guidelines when diagnosing a patient with cSCC. Poor prognosis in patients with a high metastatic potential makes cSCC clinically problematic. Clinician emphasis should be put on risk factors, anatomical site, tumor presentation, and histological features when evaluating cSCC's metastatic potential. In this review, specific skin conditions that predispose to cSCC and discrepancies in its reported metastatic potential will be discussed.

Malignant blue nevus with lymph node metastases.

BACKGROUND: Malignant blue nevi arise within cellular blue nevi and contain atypical mitoses, necrosis, nuclear pleomorphism and prominent nucleoli. Malignant blue nevus has been described as a distinct identity, a rare form of malignant melanoma, and a misdiagnosed melanoma. METHODS: We present a patient with metastatic malignant blue nevus and studies on the histopathologic, immunohistochemical, and molecular features of the neoplasm. RESULTS: Histology showed a malignant blue nevus arising in a combined intradermal and cellular blue nevus. CD117 (c-kit) staining showed diffuse cytoplasmic expression within the cellular blue nevus, decreased staining in the malignant component, and variable positivity within the lymph node metastases. Molecular loss of heterozygosity analysis showed different allelic patterns at the hOGG-1 locus between the melanoma and control skin specimens with a varying heterozygous allelic pattern in both the benign and malignant blue nevus. CONCLUSIONS: Our case of malignant blue nevus with lymph node metastasis involved mutation of the hOGG-1 DNA repair gene. CD117 showed decreased staining of the primary malignant blue nevus with marked upregulation in the metastatic lesion, unlike most metastatic melanomas. Further study is needed to determine if hOGG-1 mutation or c-kit upregulation play a role in the pathogenesis of dendritic melanocytic lesions (either benign or malignant).

Juvenile hyaline fibromatosis: a case report and review of the literature.

BACKGROUND: Juvenile hyaline fibromatosis (JHF) is a rare, inherited condition characterized by tumor-like growth of hyalinized fibrous tissue on the head and neck, joint contractures, and gingival hypertrophy. There may be marked clinical heterogeneity. METHODS: We present a case of a 3-year-old Haitian boy with multiple firm nodules on the scalp and chin without joint contractures or gingival hypertrophy. Family history was not available. RESULTS: Biopsy specimens from three scalp nodules were processed with routine and immunohistochemical stains. The matrix was periodic acid Schiff (PAS) and Alcian blue positive. The cellular stromal component was positive for vimentin and scattered factor XIIIa positive cells were found. Osteoclast-like giant cells were also noted, and stained for CD68. CONCLUSIONS: Our patient had the nodular growths on the scalp and face that are characteristically found in JHF. Microscopic examination confirmed the diagnosis and showed scattered intracytoplasmic and extracellular eosinophilic globules in three separate biopsy specimens. These were positive with PAS.

Epidermal basaloid proliferation in cutaneous myxomas.

BACKGROUND: Basaloid epidermal proliferations, which histologically resemble basal cell carcinoma, have been described overlying dermatofibromas. Several etiologies have been proposed. Cutaneous myxomas are also benign mesynchymal tumors. PURPOSE: Basaloid proliferations have been noted overlying cutaneous myxomas. We have undertaken a study to attempt to differentiate whether these are basal cell carcinomas or benign basaloid proliferations. METHODS: Thirty cases of cutaneous myxomas were included in this study. The lesions were stained with hematoxylin-eosin and alcian blue. Immunohistochemical staining for both epidermal growth factor receptor (EGF-r) and p53 protein was performed on the cutaneous myxomas with epidermal basaloid proliferation. RESULTS: Of the 30 cases of cutaneous myxomas, nine were found to have an associated overlying basaloid proliferation. The basaloid proliferations were limited to the epidermis overlying the myxoid changes within the dermis. Mitotic figures were rare. Staining for p53 protein showed scattered positive staining in the basal cells in both the basaloid proliferations and adjacent epidermis. EGF-r showed positive staining of the overlying epidermis and basaloid proliferation in five cases. CONCLUSIONS: We report basaloid proliferations overlying cutaneous myxomas and propose that these represent benign adnexal proliferations rather than superficial basal cell carcinoma and are analogous to the basaloid proliferations overlying dermatofibromas.

Familial anetoderma.

A 31-year-old Caucasian male presented with a history of erythematous, saccular outpouchings of the skin on his back, shoulders, and upper extremities (Fig. 1). The patient reported that his mother and aunt had a similar skin disorder, which initially began with inflammation, but healed leaving lax skin in its wake. He did not recall the name of the skin condition. Physical examination revealed large confluent zones of sac-like protrusions of erythematous skin on the back and shoulders. Histopathologic examination of the excisional biopsy revealed a relatively unremarkable epidermis. Perivascular lymphocytes were present in small numbers in the papillary dermis. Adnexal structures and deep dermis remained intact. The acid-orcein-Giemsa stain highlighted the absence of elastic fibers within the mid and lower reticular dermis (Fig. 2a,b), consistent with anetoderma.

Metastatic carcinoid to the conducting system-is it a rare or merely unrecognized manifestation of carcinoid cardiopathy?

We report a unique case of carcinoid cardiopathy. The patient, a 59-year-old white woman with a known carcinoid tumor of the terminal ileum, presented with complete heart block and subsequently died. Because of her clinical presentation, we examined the conducting system at autopsy. The atrioventricular node was heavily infiltrated by metastatic carcinoid. The tumor was also seen in the surrounding atrial myocardium. No other metastatic lesions were seen in the heart. Carcinoid cardiopathy in the form of right-sided endocardial and valvular thickening has been well documented in the literature, and we found these features in our case as well. To our knowledge, carcinoid has never been documented in the cardiac conducting system until now. We found 2 cases of possible involvement of the conducting system reported in the literature. Unfortunately, the conducting system was not examined postmortem in either of these cases. If conducting system involvement is not unique to the present case, patients with metastatic carcinoid and cardiac problems may require different management. We hope that this case will stimulate further postmortem study of the conducting system in patients with metastatic carcinoid tumor.

Activation of the alternative pathway of human complement by haemoglobin

Haemoglobin solutions (concentration >1.5mg/ml), prepared from lysates of erythrocytes from a normal subject and from a patient with sickle cell anaemia, caused factor B and C3 cleavage and loss of haemolytic activity of factor B when incubated with fresh autologous serum. Under the same experimental conditions, preparations of erythrocyte stroma or of buffy coat lysates did not produce factor B and C3 cleavage. This reaction required Mg++ but not Clq or C4, indicating that the alternative complement pathway was activated (Summary)

Complement activation in asymptomatic patients with sickle cell anaemia

Previous reports have suggested that a defect in serum complement may contribute to the increased susceptibility to infection shown by patients with sickle cell anaemia (SCA). In order to define the nature of any complement abnormality in SCA, we investigated the complement system in eighty-seven patients during asymptomatic periods, and analysed factor B turnover in a small sample. In these patients geometric mean serum concentrations of functionally active factor B and factor D, and of C3 and C4 protein (expressed as a percentage of normal reference serum) were lower than in controls (78 percent vs. 107 percent, P<0.001, 86 percent vs. 103 percent, P<0.001, 91 percent vs. 100 percent, P<0.01, 89 percent vs. 105 percent, P<0.05 respectively). The ratio of the serum concentration of functionally active factor B to factor B protein was lower in patients than in controls (mean 75 percent s.d 16 percent vs. mean 93 percent, s.d 22 percent P<0.001), indicating a functional deficiency of factor B protein. In addition, the fractional catabolic rate of radiolabelled factor B was markedly increased in four out of seven asymptomatic patients studied, and was inversely related to the functional factor B concentration in serum (r=-0.59, P<0.05); factor B synthesis was uniformly increased. Complement activation was not related to the presence of circulating Clq binding material. We conclude that complement activation, rather than defective synthesis as previously suggested, contributes to the abnormalities in complement component concentration and function in asymptomatic subjects with sickle cell anaemia (Summary)