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Biomedical subjects

Sigrid Regauer

Publications and source records attributed to Sigrid Regauer.

At least 19 recordsLinked to original sources

Estrogen sulfotransferase expression in endometrial stromal sarcomas: an immunohistochemical study.

Levels and activity of the enzyme estrogen sulfotransferase (EST) have been reported to play an important role in the regulation of in situ estrogen levels in human breast, prostate, and endometrial cancer. To better understand growth progression in endometrial stromal sarcomas (ESS), estrogen-dependent tumors, we analyzed the expression of EST in a series of 29 ESS. Archival formalin-fixed, paraffin-embedded material was analyzed immunohistochemically with a monoclonal antibody to EST using the peroxidase-antiperoxidase method. A score was calculated based on the percentage of positive tumor cells and staining intensity. EST staining was identified in 5 of 29 (18%) ESS and was scored as weakly positive, while 24 of 29 (82%) ESS were negative for EST. We demonstrate that a subset of ESS express EST, which may allow them to inactivate intratumoral estrogens. ESS without EST expression may have increased levels of biologically active estrogen, which explains the tumor progression of this entity and these patients may be at increased risk for recurrences and metastases.

Adult↗

Skin transplantation to monitor clinical donor-related tolerance in mixed hematopoietic chimerism.

Mixed hematopoietic chimerism usually carries with it the tolerance to any other tissue from the same donor. Consequently, the establishment of a sustained chimerism may allow long-term acceptance of transplanted organs without immunosuppression. We report a girl with refractory severe aplastic anemia who developed low recipient level hematopoietic chimerism following transplantation of maternal highly purified CD34+ cells without prophylactic immunosuppression. Renal thrombotic microangiopathy led to chronic renal failure and she received skin allografts from her mother in view of a future kidney donation. The maternal skin grafts were accepted without immunosuppression and the hematopoietic chimerism remained stable. Skin transplantation may be a helpful and easily applicable tool to monitor donor-related tolerance in hematopoietic chimerism clinically. It should contribute to minimize the risks of subsequent solid organ transplantation from the same donor without immunosuppression.

Anemia, Aplastic↗

Fetal microchimerism in Hashimoto's thyroiditis: a quantitative approach.

OBJECTIVE: Fetal microchimerism (MCH) has been implicated in the etiology of autoimmune diseases such as autoimmune thyroiditis. The goal of the study was to reliably estimate the number of fetal engrafted cells and to further investigate factors influencing the development of MCH. METHODS: Quantitative real-time PCR amplification using Y-chromosome specific (DYS14) and autosomal (beta-globin) loci was performed on thyroid gland specimens. Furthermore, we compared the distribution of ABO and rhesus systems in mothers with and without blood MCH in relation to the blood groups of the children. RESULTS: MCH was detected in eight of 21 Hashimoto patients in a frequency range of 15 to 4900 male cells per 100,000 total cells (median 97 cells), but in none of 17 healthy thyroid glands. In a third group, consisting of 18 nodular goiters, only one sample was positive (182 male cells/100,000 total cells). No woman who had not had a prior pregnancy with a male fetus showed MCH. Mothers both with and without MCH showed the same rate of mother/child incompatibilities for the ABO and rhesus systems. CONCLUSIONS: The percentage of microchimeric cells varies to a great extent in Hashimoto's thyroiditis, and this phenomenon can occur in nodular goiter in rare instances, but it appears to be absent from normal thyroid glands. Nevertheless, the biological significance of MCH remains unclear. Moreover, we have concluded that the tested blood group systems (as opposed to their role in graft vs host disease after transplantations) have no effect on fetal MCH.

ABO Blood-Group System↗

Immune dysregulation in lichen sclerosus.

Lichen sclerosus (LS) is a chronic localized lymphocyte-mediated dermatosis of genital skin with a presumed autoimmune origin. LS is characterized by localized dense lymphocytic tissue infiltrates, vasculitic processes and extensive tissue destruction. The lymphocytic infiltrate of LS biopsies contains between 1.4% and 21% of T-cells with monoclonally rearranged T-cell receptor gamma-chain gene, and the immunophenotype is dominated by B-cells, CD4-positive T-cells and antigen-presenting dendritic cells. Antigen-driven selection of T-cells and restricted T-cell receptor usage reflects prolonged exposure of the host immune system to a local (putative LS-associated) antigen. It is presently unclear at which time point in the evolution of LS the T-cell clones emerge. All investigators of LS agree on the non-neoplastic nature of the infiltrate. However, a small percentage of LS patients show serological (systemic) evidence of T-cell immune deficiencies. The lack of long-term follow up of patients with known monoclonally rearranged T-cell receptor gamma-chain gene in their LS biopsies, however, defers a final judgement on the clinical significance of our observations.

Antibody Formation↗

p185HER2 overexpression and HER2 oncogene amplification in recurrent vulvar Paget's disease.

We evaluated p186Her2 overexpression and HER2 oncogene amplification in recurrent vulvar Paget's disease. We identified six patients with recurrent vulvar Paget's disease in our archives. The number of recurrences ranged from 1 to 11 over a time period of 1-168 months. Recurrences were evaluated immunohistochemically for p185Her2 overexpression with the HercepTest and for HER2 oncogene amplification with fluorescence in situ hybridization. p185Her2 overexpression was scored as 3 in two patients, as 2 in two patients, and as 1 in two patients. All patients with scores 2 and 3 showed HER2 oncogene amplification. Overexpression of p185Her2 and HER2 oncogene amplification appears to be common in recurrent vulvar Paget's disease.

Aged↗

Gonadotropin-releasing hormone receptor expression in endometrial stromal sarcomas: an immunohistochemical study.

Gonadotropin-releasing hormone and its receptors have been identified in several human malignancies. We evaluated gonadotropin-releasing hormone receptor expression in 30 primary and recurrent endometrial stromal sarcomas. Archival formalin-fixed and paraffin-embedded material was analyzed immunohistochemically with antisera to gonadotropin-releasing hormone receptor type I and gonadotropin-releasing hormone receptor type II using the peroxidase-antiperoxidase method. Gonadotropin-releasing hormone receptor types I and II were demonstrated in most primary endometrial stromal sarcomas in varying intensity and percentage (range, 10-100%). The staining pattern was either diffuse cytoplasmic or granular/vesicular in perinuclear distribution. Recurrences stained stronger than primary tumors. The demonstration of gonadotropin-releasing hormone receptors I and II expression in endometrial stromal sarcomas may be a rationale for a clinical study of gonadotropin-releasing hormone analogs in the treatment of women with endometrial stromal sarcomas.

Female↗

Vulvar trichogenic tumors: a comparative study with vulvar basal cell carcinoma.

Trichogenic tumors are very rare in genital skin and often cause diagnostic problems because they are mitotically active and they share some histologic features with basal cell carcinomas (BCCs). We present the clinical and histologic features of 16 vulvar trichogenic tumors (6 plaque-like, 10 nodular; average age, 65 years) in comparison with 16 BCCs (11 plaque-like, 5 nodular; average age, 78 years). All trichogenic tumors, except 1 case with HSV infection, were nonulcerated tumors. Superficial plaque-like trichogenic tumors featured basal keratinocyte proliferations with peripheral nuclear palisading but no clefting at the epithelial-stromal interface. Nodular trichogenic tumors consisted of solid lobules of squamous cells and anastomosing cords and reticulations of follicular germinative cells with mitoses and apoptosis. Large pink cells with trichohyaline granules and melanocytes resembling the inner hair sheath, and clear cells resembling the outer root sheath were common. Most cysts were keratinized, but some fluid-filled cysts showed apocrine and sebaceous differentiation. The well-defined mesenchymal component of trichogenic tumors was pale and mucinous, and contained fibrocytes and fibrillary collagen bundles. All BCCs showed surface ulcerations and clefting at the stromal-epithelial interface. BCCs showed no trichogenic differentiation and lacked an organized mesenchymal tumor component. The tumor stroma of BCCs was paucicellular, mucinous, or granulation tissue-like with an inflammatory infiltrate.

Adult↗

Mucinous carcinoid as an unusual manifestation of endodermal differentiation in ovarian yolk sac tumors.

We present, for the first time, two yolk sac tumors (YST) in women 37 and 18 years of age, one with a typical parietovisceral pattern and the other with a glandular pattern, which were associated with extensive areas of mucinous carcinoid (MC). The tumor in the first case had numerous nodules of tubulopapillary YST that merged with well-differentiated MC. This patient responded well to chemotherapy. The tumor in the second case consisted of an AFP-positive glandular YST, with a glandulopapillary pattern closely resembling fetal lung type adenocarcinoma, coexisting with an AFP-negative, cytokeratin 20-positive, atypical MC; transitional areas between the two components were also seen. In the material from the recurrences and metastases; however, no YST was present, the atypical MC having become the predominant component including areas that had become carcinomatous. There was a poor response to various chemotherapeutic regimens. AFP levels became negative during the course of disease paralleling the disappearance of the YST component and the overgrowth of an increasingly anaplastic MC. The patient died 1 year after diagnosis. We think that, in these cases, MC represented an unusual form of endodermal differentiation of the YST. It is important to differentiate the yolk sac and carcinoid components due to their different responses to chemotherapy and to evaluate the possibility of mucinous carcinoid developing into a highly aggressive carcinoma.

Adolescent↗

Aromatase expression in low-grade endometrial stromal sarcomas: an immunohistochemical study.

Aromatase expression has been described in stromal cells of endometriosis, adenomyosis and endometrial cancer. We analyzed aromatase expression in a series of 23 low-grade endometrial stromal sarcomas. Archival formalin-fixed and paraffin-embedded material was analyzed with immunohistochemistry. Aromatase expression was evaluated with a monoclonal and a polyclonal antibody using the peroxidase-antiperoxidase method. A score was calculated based on the percentage of positive tumor cells and the staining intensity. Aromatase was seen in 19 (83%) of 23 tumors with monoclonal antibody and 20 (87%) of 23 tumors with polyclonal antibody. Aromatase expression using the monoclonal antibody was scored as high in five (22%), moderate in nine (39%) and low in five (22%) tumors. Four (17%) low-grade endometrial stromal sarcomas did not stain for aromatase. Aromatase expression with the polyclonal antibody was scored as high in seven (31%), moderate in four (17%) and low in nine (39%) tumors. Three (13%) low-grade endometrial stromal sarcomas did not stain for aromatase. Little or no aromatase expression tended to correlate with stage I disease, while higher scores were more frequently associated with advanced disease. Our results demonstrate that most low-grade endometrial stromal sarcomas express aromatase. The staining pattern, however, is heterogeneous. The high percentage of aromatase positivity in low-grade endometrial stromal sarcomas may have implications in the management of these tumors and offer new treatment modalities such as hormonal therapy with aromatase inhibitors.

Adult↗

Penile clear cell carcinoma: a report of 5 cases of a distinct entity.

We present a series of 5 penile clear cell carcinomas, which arose in middle-aged men at the inner side of the foreskin. They were large, exophytic, partly ulcerated, and widely invasive tumors with sharp demarcation to the surrounding normal skin/mucosa. Histologically, they were composed of large clear cells with intracytoplasmic PAS/d-PAS-positive material and showed extensive lymphatic and blood vessel invasion. Strong staining with antibodies to Muc-1, EMA, and CEA was typical. All carcinomas harbored HPV16 DNA, although only one carcinoma revealed HPV-related cytologic cell changes. All 5 patients had extensive, partly cystic inguinal lymph node metastases with a striking clear cell differentiation and focal dense sclerotic basement membrane material, either at or within several months after initial diagnosis. Two patients are alive without disease after 7 and 10 years. One patient died after 9 months of widespread disease and 2 patients are presently alive at 7 and 17 months follow-up with widespread lymphatic and hematogenous metastases despite adjuvant chemo- and radiation therapy. In contrast to squamous cell carcinoma, penile clear cell carcinomas show extensive blood and lymph vessel invasion and early metastases to regional lymph nodes. Clear cell carcinomas represent a distinct group of penile cancers that may have a different clinical behavior than usual penile squamous cell carcinomas.

Adenocarcinoma, Clear Cell↗

Recurrent verruciform xanthoma of the vulva.

Verruciform xanthoma is a rare, benign, mucocutaneous, nondestructive lesion characterized by proliferation of non-Langerhans lipid-rich histiocytes. We describe the clinical and pathologic findings in a 30-year-old female with recurrent verruciform xanthoma of the vulva 8 years after initial therapy. The differential diagnosis includes seborrheic keratosis, verruca simplex, condyloma acuminatum, granular cell myoblastoma, vulvar intraepithelial neoplasia, bowenoid papulosis, erythroplasia of Queyrat, and verrucous carcinoma.

Adult↗