Congenital central corneal dermoid: A rare entity.

BACKGROUND: To understand the pathogenesis of a central corneal dermoid (CD) in a 12-day-old child, a comparison of CD specimen was done with limbal dermoid (LD) and cadaveric corneal (CC) specimens by immuno-histochemical staining. METHODS: The child underwent penetrating keratoplasty for visual rehabilitation. The corneal tissue was sent for histo-pathological and immunochemistry evaluation. The corneal specimen obtained was compared the origin of central CD with LD and CC based on their antigenic expression profile. RESULTS: Clinically over a period of 75 months post operatively the child maintained a clear graft. Hematoxylin and Eosin staining of LD had a typical morphology including stratified keratinized epithelium, hair shaft with pilo sebaceous glands, eccrine sweat glands, lymphocytes, and blood vessels. Immuno-histochemical staining showed positive stain for Cytokeratin 3 epithelial marker in the epithelium of CC, LD, and CD. Smooth muscle maker (SMA) was identified in LD and CD but not in the CC as it is devoid of blood vessels. Limbal stem cell maker (P63) was detected only in LD. Vimentin, a mesenchymal stem cell marker stained positively in all three tissues of CC, LD, and CD. CONCLUSIONS: Corneal dermoid showed positive staining for mesodermal tissue components compared to both ectodermal and mesodermal components in limbal dermoid suggesting possibly a different origin of corneal dermoid.

Keratin Granulomas in the Peritoneum on Frozen Section: A Case Report with Multiple Suspects and the Search for the Culprit.

Keratin granulomas in the peritoneum are a rare finding with multiple etiologies and can be especially challenging for both the pathologist and the surgeon when these lesions are grossly visible. We report a case of a unique frozen section diagnostic scenario of evaluation of keratin granulomas in the peritoneum of a 47-year-old woman in the setting of multiple potential culprits: endometrial endometrioid adenocarcinoma following fertility sparing treatment, and a concurrent dermoid cyst. We discuss the various etiologies of keratin granulomas in the peritoneum, mechanism of their formation, diagnostic significance, as well as implications of fertility sparing treatments. To the best of our knowledge, this is the only case of keratin granulomas in the peritoneum with multiple distinct potential pathologic culprits as well the only case following fertility sparing treatment.

Incidental Detection of Radiotracer Uptake in Intracranial Dermoid Cyst on 18F-Prostate-Specific Membrane Antigen PET/CT During Staging for Prostate Carcinoma.

ABSTRACT: A 68-year-old man underwent 18F-prostate-specific membrane antigen (PSMA) PET/CT for staging of a newly diagnosed prostate adenocarcinoma. Unexpectedly, PET/CT revealed high focal 18F-PSMA brain uptake, which initially was suspected for a brain metastasis. Corresponding CT and MRI scans revealed characteristic imaging features of an intracranial dermoid cyst at this site. This is an exceptional location for a dermoid cyst, which had been followed up conservatively with no substantial changes. This case shows that dermoid cyst should be added to the reported list of benign neoplasms that shows "false-positive" PSMA uptake during evaluation of patients with prostate carcinoma, representing a potential interpretative pitfall.

Classification of cysts with follicular germinative differentiation.

BACKGROUND: Cysts are very common in the routine of dermatopathology but follicular germinative (trichoblastic) differentiation in cysts is seen rarely. The presence of follicular germinative differentiation in a cyst alerts to consider the possibility of a basal cell carcinoma (BCC) arising in a cyst. METHODS: Five cystic lesions with zones of follicular germinative differentiation were collected. Hematoxylin and eosin sections were reassessed for architecture, types of follicular differentiation and stromal characteristics; immunohistochemical studies with Ber-EP4 were analyzed. Articles about follicular germinative differentiation in cystic lesions were reviewed. RESULTS: Cystic lesions with follicular germinative differentiation have been described in the literature under various names including trichoblastic infundibular cyst, cystic trichoblastoma, cystic panfolliculoma (CPF), dermoid cyst with basaloid proliferations, folliculosebaceous cystic hamartoma and BCC occurring in infundibular cysts. The lesions presented by us could be classified as three cystic trichoblastomas, one CPF and one cystic hamartoma with follicular germinative differentiation. CONCLUSIONS: Histopathologically, cystic trichoblastomas can be separated from CPFs. Some lesions defy classification and may be regarded as cystic follicular hamartomas. The presence of follicular papillae and bulb-like structures, advanced follicular differentiation like that of inner and outer root sheath exclude the differential diagnosis of BCC arising in a cyst.

The expression of cytokeratin in keratocystic odontogenic tumor, orthokeratinized odontogenic cyst, dentigerous cyst, radicular cyst and dermoid cyst.

The epithelial lining of odontogenic keratocysts exhibits either parakeratosis or orthokeratosis. In 2005, the WHO classified odontogenic keratocysts with parakeratosis as keratocystic odontogenic tumors (KCOT). Odontogenic keratocysts with orthokeratosis were not classified as odontogenic tumors, but instead referred to as orthokeratinized odontogenic cysts (OOC). To clarify the difference between these two lesions, we investigated their biological characteristics using immunohistochemical studies for cytokeratins (CK) in KCOT and OOC as well as in dentigerous cysts (DC), radicular cysts (RC) and dermoid cysts (DMC). We examined twenty-five cases of KCOT, fifteen cases each of OOC, DC and RC, and ten cases of DMC. We studied the immunohistochemical expression of CK10, 13, 17 and 19. To evaluate the immunohistochemical staining pattern, we divided the epithelial lining of the lesions into three layers (surface layer: su, spinous layer: sp, basal layer: ba). For CK10, most OOC and DMC specimens of su and sp were positive. For CK13 and 19, most KCOT, DC and RC specimens of su and sp were positive. For CK17, most KCOT specimens of su and sp were positive. The percentages of total CK expression of su and sp, and ba of CK19 differed significantly between the lesions (P < 0.001). These results support the hypothesis that OOC originate from not the odontogenic apparatus, but the oral epithelial component.

Limbal dermoid epithelium shares phenotypic characteristics common to both hair epidermal and limbal epithelial stem cells.

PURPOSE: To determine putative limbal epithelial stem cell marker expression in human limbal dermoids compared to stem cell niches in normal limbus and hair follicles of normal human dermis. METHODS: Human limbal dermoids (n = 7), normal skin (n = 2) and normal limbal (n = 7) tissue were examined. Immunohistochemistry was performed on paraffin embedded specimens using automated and manual immunostaining with primary antibodies to CK15, CK14, Cadherin-P (CDH3), Wnt-3, Wnt-4, Wnt-5a, Dickkopf (DKK)-3, Sox-2, Sox-10, Sox-13, PEDF, NGFR p75 and ß-catenin. RESULTS: Positive immunostaining was found for CK15, CK14, CDH3, NGFR p75, PEDF, Sox-2, Sox-10 and Wnt 4 in the basal dermoid epithelium, limbus and hair follicles. Suprabasal epithelium was immunostained with PEDF, Sox-2 and Wnt-4 in these tissues. The sebaceous and sweat glands, vascular endothelium and nerves of the limbal dermoid immunostained with PEDF and Sox-2. Sebaceous and sweat glands stained for Sox-10. DKK-3 immunostaining occurred in the dermoids' suprabasal epithelium and vascular endothelium but not in the limbus or hair follicle. CONCLUSION: Human limbal dermoids share a similar antigenic expression profile similar to the basal limbal epithelium and to the stem cell niche of hair follicles. This supports the notion that limbal dermoids could have properties in common with limbal and/or dermal epithelial stem cells.

Increased levels of oxidative stress markers in the peritoneal fluid of women with endometriosis.

OBJECTIVE: To evaluate 8-hydroxy-2-deoxyguanosine (8-OHdG) and 8-isoprostane levels in the peritoneal fluid (PF) of women with endometriosis. STUDY DESIGN: One hundred and ten women with laparoscopically and histopathologically confirmed endometriosis and, as reference groups, 119 patients with simple serous (n=78) and dermoid (n=41) ovarian cysts were studied. Peritoneal fluid 8-OHdG and 8-isoprostane concentrations were evaluated by enzyme-linked immunosorbent assays. RESULTS: 8-OHdG and 8-isoprostane levels in peritoneal fluid were significantly higher in patients with endometriosis compared with the reference groups. Higher PF 8-OHdG and 8-isoprostane concentrations were observed in patients with advanced stages of endometriosis. A statistically significant positive correlation was found between 8-OHdG and 8-isoprostane levels in peritoneal fluid. CONCLUSION: Endometriosis induces greater oxidative stress and frequent DNA mutations in peritoneal fluid than nonendometriotic ovarian cysts. The most severe oxidative stress occurs in the peritoneal cavity of women with more advanced stages of the disease.

Interleukin-6-producing dermoid cyst associated with multicentric Castleman's disease.

Dysregulated overproduction of interleukin-6 (IL-6) from activated B cells in affected lymph nodes has been implicated in the pathogenesis of multicentric Castleman's disease (MCD), a rare lymphoproliferative disorder accompanied by systemic manifestations. We here report the case of a 32-year-old female presenting with MCD associated with a dermoid cyst in the pelvic cavity. The co-occurrence of MCD and dermoid cyst has not been reported before. Immunohistochemical analysis of the tissue sections showed IL-6 production in CD68-positive macrophage cells, which had infiltrated the dermoid cyst. Removal of the cyst resulted in partial improvement in systemic symptoms accompanied by a decrease in serum IL-6, while complete improvement was obtained by treatment with an anti-IL-6 receptor antibody following resection of the dermoid cyst. To the best of our knowledge, this is the first study to provide evidence of IL-6 production by CD68(+) cells in a dermoid cyst involved in MCD.

Proteomic analysis of mesenchymal stem-like cells derived from ovarian teratoma: potential role of glutathione S-transferase M2 in ovarian teratoma.

Ovarian teratoma is a dermoid cyst in the ovary that contains mature tissues such as hair, teeth, bone, thyroid, etc. To understand the molecular mechanisms of ovarian teratoma growth, a comparative proteomic analysis was undertaken using mesenchymal stem cell-like cells (MSCLCs) isolated from normal human ovarian or teratoma tissues. Both normal ovarian and teratoma MSCLCs expressed stem cell markers OCT4 and NANOG, and were negatively staining with the senescence-associated (SA) ß-galactosidase. Furthermore, teratoma MSCLCs had higher proliferation and colony formation rates, with more angiogenic property than that of normal MSCLCs. Proteomic study revealed that 17 proteins had the expression changes over eightfold in ovarian teratoma MSCLCs compared with normal control. Interestingly, among them, GSTM2 was strongly expressed in teratoma MSCLCs. Moreover, overexpressed GSTM2 in the teratoma was associated with downregulation of p38 MAPK and activation of AKT and survivin. Taken together, these findings suggest that that ovarian teratoma MSCLCs have a higher potency for proliferation and angiogenesis and GSTM2 appears to be involved in the regulation of other survival genes.

[Rupture of intracranial dermoid cyst with disseminated lipid droplets]. / Dissémination sous-arachnoïdienne de gouttelettes lipidiques par rupture d'un kyste dermoïde intracérébral.

INTRODUCTION: Dermoid cysts are rare slow-growing benign tumors of the central nervous system generally diagnosed in the third to fifth decade. They are formed from inclusion of ectodermal elements during neural tube closure, and are mostly located along the cranial or spinal midline axis. They cause many non specific symptoms such as headache and seizures, and may spontaneously rupture spreading fatty droplets into the ventricles and subarachnoid spaces. Rupture of dermoid cysts causes sequelae which may vary from no symptoms to death. In general, subtotal surgical removal is required for ruptured dermoid cysts. CASE REPORTS: We report two cases of ruptured intracranial dermoid tumor with non-specific clinical presentations. The first rupture was asymptomatic and discovered on brain magnetic resonance imaging (MRI) performed for other purposes. The second case was identified on brain imaging performed because of daily headache. These dermoid cysts were not surgically treated. Surveillance was advised because of the spontaneously favourable outcome observed in both cases. CONCLUSION: Surgical removal is not the only treatment of ruptured dermoid cyst. Monitoring with brain MRI can be sufficient if the rupture has no severe clinical impact.

Virilizing ovarian dermoid cyst with leydig cells.

BACKGROUND: The clinical observation of virilization is a rare finding that has a number of possible explanations. Overall, ovarian tumors causing virilization are exceedingly rare and mostly occur in post-menopausal women. In fact, there are no reported cases of virilization from a testosterone-producing ovarian dermoid in the adolescent female age group. The most frequent germ cell tumor derived from the ovaries is the benign cystic teratoma (dermoid) which accounts for 25% of all ovarian neoplasms. Teratomas consist of tissues that recapitulate the ectoderm, endoderm, and mesoderm. Usually the tumors are asymptomatic, but they occasionally can cause severe pain if there is torsion or if sebaceous material perforates the cyst wall, leading to reactive peritonitis. CASE: A 12-year-old female was found to have a large 3 5 x 19 x 12 cm ovarian mature cystic teratoma arising from her right ovary. The patient also displayed evidence of masculinization demonstrated by a deepening voice and clitoromegaly. The dermoid was producing large amounts of testosterone from a nest of Leydig cells found pathologically in the mass. CONCLUSION: Benign cystic teratomas can produce active hormones, albeit rarely. This is a finding important to consider when ovarian cystectomy is performed for removal of a benign cystic teratoma.

Metabolic changes in pelvic lesions: findings at proton MR spectroscopic imaging.

OBJECTIVE: The purpose of this study is to investigate the in vivo magnetic resonance spectroscopic (MRS) features of pelvic lesions using long echo time and to characterize the spectral patterns of various pathological entities. MATERIALS AND METHODS: 17 patients with surgically and histopathologically confirmed pelvic lesions underwent long echo-time MRS, and the results obtained were analyzed. Before laparotomy, choline (Cho), lactate, lipid and creatine (Cr) levels of all lesions were measured by single voxel MRS (point-resolved spectroscopy technique, TE 136 ms). Voxels were placed in the center of the lesions. The MRS results of lesions were compared with the final histopathological diagnoses. RESULTS: Spectroscopy analysis of serous, mucinous and undifferentiated carcinoma of the ovary revealed Cho, lactate and lipid signals, but granulosa-theca cell tumor showed only a lipid signal. The Cho signal was obtained from only 3 patients with mature cystic teratoma but none of the other benign ovarian tumors and pelvic abscesses. A lipid signal was detected in 3 patients diagnosed with pelvic abscess and all benign ovarian tumors. In addition to the lipid signal, a lactate signal was detected in the spectra of two pelvic abscesses. One case of endometrioma and 1 case of teratoma did not show any signal. CONCLUSION: MRS demonstrates significant differences in metabolite concentration between benign and malignant ovarian tumors and pelvic abscesses. MRS may therefore be helpful in the differential diagnosis of adnexal lesions.

In vivo proton magnetic resonance spectroscopy of human spinal mass lesions.

OBJECTIVE: To observe metabolic differences between spinal tumor and other diseases in human spinal mass lesions, in vivo 1H magnetic resonance spectroscopy (MRS) was attempted to obtain metabolic signals in patients with various spinal mass lesions. METHODS: 1H nuclear magnetic resonance (NMR) spectra were obtained from 14 patients before surgery using a receive-only surface coil on a 1.5 T clinical magnetic resonance imaging (MRI) unit. MRS findings were compared with the histopathologic results from biopsy. In addition, tumor spectra were compared with the spectra of other benign diseases including disc herniation, which can mimic spinal cord tumor. In vitro 1H-NMR spectra were also collected from perchloric acid extracts of some spinal tumors. RESULTS: Typical water resonance line widths were in the 6- to 10-Hz range, but the metabolic signals observed were sufficiently resolved to be assigned from comparison with the 1H spectra of brain tissue. Choline was detected in all tumor spectra (n = 6) except ependymoma, whereas it was absent in other benign diseases including disc herniation (mimicking spinal cord tumors), dermoid cyst, tuberculosis, and non-multiple sclerosis myelitis. Spectral patterns of meningiomas, schwannomas, metastasis from renal cell carcinoma, and ependymomas in the spinal cord were similar to those of central nervous system (CNS) tumors. It was not possible to observe distinctive metabolic differences between benign diseases owing to relatively larger line broadening of some signals compared with that in CNS tissue. CONCLUSIONS: It appeared that acquisition of in vivo 1H-NMR signals was possible in human spinal mass lesions on a 1.5 T clinical MRI unit. Detection of choline only in the spinal tumors may indicate that there is some potential in using in vivo 1H-MRS to distinguish spinal tumors from disc herniation mimicking spinal cord tumors, non-multiple sclerosis myelitis, and dermoid cysts. On the basis of our NMR findings, however, it was not possible to distinguish between benign diseases.

Investigation of glutathione concentrations in peritoneal fluid from women with and without endometriosis.

UNLABELLED: Changes in the peritoneal fluid (PF) environment have been implicated in the pathogenesis of endometriosis as well as in the decrease of fertility. OBJECTIVE: To evaluate the concentration of glutathione in PF of women with endometriosis. PATIENTS: Twenty-one patients with endometriosis (I or II rAFS stage, n=11; III or IV rAFS stage, n=10), and 29 patients with follicular or dermoid ovarian cysts (n=17 and n=12, respectively). RESULTS: Mean (+/-S.D.) PF glutathione concentration was 0.22+/-0.01 micromol/ml in patients with minimal or mild endometriosis, 0.21+/-0.05 micromol/ml in women with III or IV stage of the disease, 0.24 +/- 0.03 micromol/ml in women with follicle ovarian cysts, and 0.23+/-0.05 micromol/ml in patients with dermoid tumors of ovaries. No significant difference in the peritoneal glutathione level was found between the groups. CONCLUSION: These results suggest that PF glutathione is not involved in the progression of endometriosis.

Virilizing ovarian dermoid cyst with peripheral steroid cells. A case study with immunohistochemical study of steroidogenesis.

A case of virilizing ovarian dermoid cyst with peripheral steroid cells and virilization is reported in a 62-year-old woman. The level of testosterone dropped to normal after oophorectomy. The cyst wall was bordered by a discontinuous band of steroid cells focally accompanied by smooth muscle cells. Immunohistochemically, the steroid cells were enzymatically active and displayed a profile similar to the internal theca cells of ovarian follicles. These steroid cells were most probably modified stromal cells associated with smooth muscle metaplasia of the ovarian stroma.

Soluble adhesion molecules in serum and cyst fluid from patients with cystic tumours of the ovary.

The potential of the soluble forms of the adhesion molecules ICAM-1 (sICAM-1), CD44std (sCD44std) and E-cadherin (sE-cadherin) was tested for the diagnosis of benign and malignant cystic epithelial tumours of the ovary. Concentrations of sICAM-1, sCD44 std and sE-cadherin were measured by enzyme-linked immunosorbent assay (ELISA) in the serum and cyst fluid obtained from 23 patients with luteal cysts, 29 with cystadenomas, nine with dermoid cysts, five with borderline tumours and 11 with carcinomas. Serum concentrations of sICAM-1, but not of sCD44std and sE-cadherin, were constantly elevated compared with normal controls. Cyst fluid concentrations of sICAM-1, sCD44std and sE-cadherin were elevated in borderline and malignant tumours compared with cystadenomas (P = 0.034, 0.006 and 0.001, respectively). In conclusion, our results suggest that serum concentrations of adhesion molecules have no diagnostic value in ovarian tumours, whereas cyst fluid concentrations may facilitate distinction between benign lesions and borderline or malignant tumours.