Xeroderma pigmentosum (XP) is a rare autosomal recessive disease characterized by hypersensitivity of the skin to ultraviolet radiation and other carcinogenic agents. This ailment is characterized by increased photosensitivity, skin xerosis, early skin aging, actinic keratosis, erythematous lesions, and hyperpigmentation macules. In this serial case report, we presented four cases with XP from two families in Indonesia. Both families were referred from rural referral health centers, and each family has two affected siblings. They had freckle-like pigmentation on the face, trunk, and extremities, which progressed since childhood. One patient of family 2 died because of an infectious disease. Histopathological examination using cytokeratine (CK), CD10, and Ber-EP4 staining from available tissue biopsy of one affected case of family 1 identified basal cell carcinoma (BCC) on the cheek and melanoma on the right eye. Mutation analysis found ERCC2, c2047C>T and XPC, c1941T>A in the first and second families, respectively. We suppose that this is the first case report of XP in Indonesia that incorporates clinical examination, genetic analysis, and extensive histopathological examination, including immunohistochemistry staining, and a novel pathogenic variant of XPC was found in the second family.
This study was conducted to explore the feasibility of culturing conjunctiva epithelial cells in serum-free and feeder layer-free culture system with regard to the cell morphology and immunocytochemistry of the rabbit bulbar, fornix and palpebral conjunctiva epithelia. The results showed that epithelium cells from all the three conjunctiva regions can be cultured in a serum-free and feeder layer-free environment. We obtained highest epithelial growth from fornix region with minimum invasion of fibroblast cells compared to other area. All cultured cells were stained positive for cytokeratin 19 and MUC5AC and negative for cytokeratin 3. These findings suggested that fornix was a better source of cells for the development of tissue engineered conjunctiva for future clinical application.
Sixty-two "leukoplakias" from the cheeks of betel-nut chewers in West Malaysia were studied histologically. Ten biopsies were from non-tobacco betel-nut chewers. An amorphous von Kossa positive layer was seen on the keratin surface in 42 specimens. Tobacco did not appear essential for its formation, and it appeared to be significantly associated with parakeratosis. Its possible significance as a cuticle-like layer prolonging contact between carcinogens and the mucosa is discussed.Parakeratosis appeared to be the most common form of cornification seen, and the mitotic activity in parakeratinized leukoplakias appeared to be significantly greater than orthokeratinized leukoplakias.Comparison with studies on other population samples using different quids suggested that severe histological changes were more likely to be seen when tobacoo-containing quids were chewed as compared to non-tobacco-containing quids.An attempt to correlate the histological changes seen with the clinical habit in leukoplakias from chewers using tobacco-containing quids suggested that epithelial atrophy appeared to be significantly related to the duration of the habit but not to the "intensity" of the habit.
Mature cystic teratoma is the commonest ovarian germ cell tumour which accounts for 70% of all benign ovarian neoplasms in the reproductive age groups. Being a pluripotent germ cell tumour, mature cystic teratoma has at least two out of three mature embryonic germ cell components: ectoderm, mesoderm and endoderm. The presence of multiple cystic spaces within the tumour wall, also known as pneumatosis cystoides-like appearance is rarely described but a characteristic feature in cystic teratoma of the ovary. Currently, there is little information concerning the mechanism of its formation. Herein, we described an unusual case of ovarian mature cystic teratoma in a 31-year-old pregnant female with multiple sieve-like areas resembling pneumatosis cystoides of the intestine. Macroscopic and histological examination of the ovary revealed features diagnostic of mature cystic teratoma. Intriguingly, multiple cystic spaces of variable sizes were found within the cyst wall histologically. They were lined partially or completely by foamy histiocytes and foreign body type multinucleated giant cells, exhibiting strong CD68 immunoreactivity. Vascular endothelial markers (CD31 and CD34) and epithelial marker (cytokeratin AE1/AE3) were negative. A diagnosis of mature cystic teratoma with pneumatosis cystoides-like feature was rendered. The patient was discharged well with no signs and symptoms of early labour. The etiopathogenesis of this intriguing histological feature is briefly discussed in this article.
An adult female Sumatran rhinoceros was observed with a swelling in the left infraorbital region in March 2017. The swelling rapidly grew into a mass. A radiograph revealed a cystic radiolucent area in the left maxilla. In June 2017, the rhinoceros was euthanized. At necropsy, the infraorbital mass measured 21 cm × 30 cm. Samples of the infraorbital mass, left parotid gland, and left masseter muscle were collected for histopathology (Hematoxylin & Eosin, Von Kossa, Masson's trichrome, cytokeratin AE1/AE3, EMA, p53, and S-100). Numerous neoplastic epithelial cells showing pleomorphism and infiltration were observed. Islands of dentinoid material containing ghost cells and keratin pearls were observed with the aid of the two special histochemistry stains. Mitotic figures were rarely observed. All the neoplastic odontogenic cells and keratin pearls showed an intense positive stain for cytokeratin AE1/AE3, while some keratin pearls showed mild positive stains for S-100. All samples were negative for p53 and S-100 immunodetection. The mass was diagnosed as a dentinogenic ghost cell tumor.
Plastics have been one of the highly valued materials and it plays an significant role in human's life such as in food packaging and biomedical applications. Bioplastic materials can gradually work as a substitute for various materials based on fossil oil. The issue like sustainability and environmental challenges which occur due to manufacturing and disposal of synthetic plastics can be conquering by bio-based plastics. Feathers are among the most inexpensive abundant, and renewable protein sources. Feathers disposal to the landfills leads to environmental pollutions and it results into wastage of 90% of protein raw material. Keratin is non-burning hydrophilic, and biodegradable due to which it can be applicable in various ways via chemical processing. Main objective of this research is to synthesis bioplastic using keratin from chicken feathers. Extracted keratin solution mixed with different concentration of glycerol (2 to 10%) to produce plastic films. The mixture was stirred under constant magnetic stirring at 60 °C for 5 h. The mixtures are then poured into aluminum weighing boat and dried in an oven at 60 °C for 24 h. The mechanical properties of the samples were tested and the physic-chemical properties of the bioplastic were studied. According to the results, Scanning Electron Microscopy test showed good compatible morphologies without holes, cavity and edge. The difference in chemical composition was analyzed using Fourier transform infrared spectroscopy (FTIR). The samples were also characterized by thermo gravimetric analysis (TGA), differential scanning calorimetry (DSC), X-Ray diffraction (XRD) to check the thermal and crystallinity properties. Other than that, bioplastic made up from keratin with 2% of glycerol has the best mechanical and thermal properties. According to biodegradability test, all bioplastic produced are proven biodegradable. Therefore, the results showed possible application of the film as an alternative to fossil oil based materials which are harmful to the environment.
Management of patients with stage II colorectal carcinomas remains challenging as 20 - 30% of them will develop recurrence. It is postulated that these patients may harbour nodal micrometastases which are imperceptible by routine histopathological evaluation. The aims of our study were to evaluate (1) the feasibility of multilevel sectioning method utilizing haematoxylin and eosin stain and immunohistochemistry technique with cytokeratin AE1/AE3, in detecting micrometastases in histologically-negative lymph nodes, and (2) correlation between nodal micrometastases with clinicopathological parameters. Sixty two stage I and II cases with a total of 635 lymph nodes were reviewed. Five-level haematoxylin and eosin staining and one-level cytokeratin AE1/AE3 immunostaining were performed on all lymph nodes retrieved. The findings were correlated with clinicopathological parameters. Two (3.2%) lymph nodes in two patients (one in each) were found to harbour micrometastases detected by both methods. With cytokeratin AE1/AE3, we successfully identified four (6.5%) patients with isolated tumour cells, but none through the multilevel sectioning method. Nodal micrometastases detected by both multilevel sectioning and immunohistochemistry methods were not associated with larger tumour size, higher depth of invasion, poorer tumour grade, disease recurrence or distant metastasis. We conclude that there is no difference between the two methods in detecting nodal micrometastases. Therefore it is opined that multilevel sectioning is a feasible and yet inexpensive method that may be incorporated into routine practice to detect nodal micrometastases in centres with limited resources.
The cytological features of a rare case of undifferentiated (embryonal) sarcoma of the liver are presented. The cytology smears showed singly dispersed polygonal and spindle cells as well as loose clusters of cells held together in myxoid material. Neoplastic cells were generally large with round, oval or lobulated nuclei. The cytoplasm was variable in amount with ill-defined borders. Occasional multinucleated cells were also present. Hyaline globules were present on sections of the cell block. Immunohistochemical studies performed showed positivity for vimentin, cytokeratin and alpha-1-antitrypsin (AAT) in the tumour cells.
This was a retrospective study of odontogenic keratocysts in people from the Singapore-Malaysian region. The purpose of this study was to present the clinicopathologic features of odontogenic keratocysts in the Oriental population and to compare these data with those from other reported studies. Biopsy records from 1981 to 1992 of 61 cases of odontogenic keratocysts from patients in Malaysia and Singapore showed that 42.6% of patients were female and 57.4% of patients were male. Among patients with cysts, 75.4% were Chinese, 6.6% were Malays, 9.8% were Indians and 8.2% were other ethnic groups. The mean age of these patients was 26.98 +/- 15.38 years with a peak incidence occurring in the second to fourth decades. The location of the lesions was more often in the mandible (65.5%) than the maxilla (31.0%). There was a marked predilection for lesions to occur in the posterior mandible. Histologically, 90.2% of the cysts were lined with a para-keratinized stratified squamous epithelium while only 3.3% of the cysts were lined with orthokeratinized stratified squamous epithelium. Mixed para-keratinized and orthokeratinized epithelial linings were observed in 4 cases (6.5%). The cyst linings were mainly uninflamed (95.1%). Inflammation of the cyst wall was found in 42 cases (68.8%). Twelve (19.7%) cases contained keratin in the lumen. A satellite cyst was observed in only 6 cases (9.8%). In conclusion, most clinical and histological features seen in this study were similar to those found for Caucasians. The only clinical feature that was different was the peak age incidence, that ranged from the second to fourth decades, with an absence of a second peak. Odontogenic keratocysts presenting at the site of the dentigerous cyst were observed in 7 cases (11.5%).
The odontogenic keratocyst has been well documented and extensively studied. It is of particular interest because of its high recurrence rate and aggressive nature. The material for this study consisted of 70 cases of odontogenic keratocysts in predominantly ethnic Chinese patients who were treated from 1981 to 1996. The cases were retrospectively studied to compare characteristics of the lesion in this population with those in previous reports. Most of the patients in this series were 21 to 30 years of age. Association with an impacted mandibular third molar was found in more than 50% of the cases. The recurrence rate was 20% for 35 patients with a follow-up period of at least 5 years. The follow-up period for the whole series ranged from 1 to 16 years. Treatment was surgical enucleation with peripheral ostectomy. There were no significant differences in characteristics with respect to presentation and prognosis between this series and those described in previous publications.
Inhibition studies were carried out to study possible cross-reactivity between a peptide fragment of the Epstein-Barr virus nuclear antigen, EBNA-1, and keratin/collagen. The 20-amino acid peptide (pAG), derived from a glycine-alanine repeat region of EBNA-1, uniquely makes up about one-third of the viral protein and is a dominant IgA antigenic epitope in patients with nasopharyngeal carcinoma (NPC). A small percentage of normal human sera (NHS) also binds pAG and this reactivity is examined in this study. Ten percent (2/20) and 13.4% (2/15) of IgA-pAG-positive NPC sera and NHS, respectively, were significantly inhibited by keratin in a competitive ELISA system. Conversely, 31.6% (6/19) and 30.8% (4/13) of IgA-keratin-positive NPC sera and NHS, respectively, were significantly inhibited by pAG. This indicated minimum cross-reactivity between IgA serum antibodies to EBNA-1 and keratin. Using collagen as inhibitor, none of 18 and only 2/13 IgA-pAG-positive NPC sera and NHS, respectively, were inhibited. In the collagen ELISA system, only 2/19 (10.5%) and 4/25 (16%) of IgA-collagen-positive NPC sera and NHS, respectively, were inhibited with pAG. Therefore, cross-reactivity with collagen was also low. IgA-pAG-positive NHS may therefore not be a false positive phenomenon, but whether it may represent an early serological profile related to NPC carcinogenesis remains to be determined.
Mice have served as an excellent model to understand the etiology of lung cancer for years. However, data regarding dual-stage carcinogenesis of lung squamous cell carcinoma (SCC) remain elusive. Therefore, we aim to develop pre-malignant (PM) and malignant (M) lung SCC in vivo using N-nitroso-tris-chloroethylurea (NTCU). BALB/C mice were allotted into two main groups; PM and M groups which received treatment for 15 and 30 weeks, respectively. Then, the mice in each main group were allotted into three groups; control, vehicle, and cancer (n = 6), which received normal saline, 70% acetone, and 0.04 M NTCU by skin painting, respectively. Histopathologically, we discovered a mix of hyperplasia, metaplasia, and dysplasia lesions in the PM group and intracellular bridge; an SCC feature in the M group. The M group was positive for cytokeratin 5/6 protein which confirmed the lung SCC subtype. We also found significantly higher (P
Cannon's disease or white sponge naevus is a relatively rare genetically determined skin disorder. It is inherited as an autosomal dominant trait that displays a high degree of penetrance and expressivity. This article describes cases of Cannon's disease in a mother and her son.
The lining epithelium of 15 cases of odontogenic keratocyst (OKC) was evaluated immunohistochemically. The peroxidase-antiperoxidase technique was applied to study the distribution of polyclonal keratin and S-100 protein while the indirect method was used to examine monoclonal vimentin and desmin reactivity. Consistent positive keratin staining was revealed in the lining epithelium of all 15 OKCs with additional intense staining in the stratum corneum. None of the cases showed vimentin or desmin reactivity within the lining epithelium elements. One of the 15 cysts studied showed positive S-100 protein staining in the nuclei of the lining epithelial cells. The pertinent literature on the immunophenotyping of the lining epithelium of OKC is reviewed.
Two cases of either peripheral odontogenic fibroma (POF) (WHO type) or peripheral ameloblastoma are reported. Their immunohistochemical characteristics were investigated in an attempt to clarify their histogenesis. The results showed that the epithelial component of this neoplasm tended to retain its distinct odontogenic character and expressed a keratin profile different from that of the overlying oral epithelium from which both cases most probably originated. The connective tissue element of these tumors was vimentin-positive and S-100 protein negative, confirming their mesodermal nature but precluding the possibility of ectomesenchymal derivation. No reactivity for desmin was noted.
Four cases of either combined occurrence of ameloblastoma and odontogenic keratocyst or a rare keratinising variant of ameloblastoma are presented. The cardinal histomorphologic characteristics are simultaneous occurrence of ameloblastomatous epithelial islands with central keratinisation and multiple keratinising cysts. Immunohistochemically the tumour elements were keratin positive and occasionally S-100 protein and desmin positive. Major differential diagnosis of these neoplasms are discussed.
Granular cell ameloblastoma (GCA) is a well recognized variant of follicular ameloblastoma with extensive granular cell change. In contrast, plexiform granular cell odontogenic tumor (PGCOT) is a rare and recently described lesion characterized histologically by a monophasic plexiform pattern of granular cells. In this paper, two cases of an unusual granular cell odontogenic tumor exhibiting combined features of these two entities are described along with their immunohistochemical characteristics. The granular cells of both the GCA and PGCOT areas showed similar patterns of expression for keratin and S-100, which differed from those of typical ameloblastoma. No reactivity for desmin or vimentin was noted. The histomorphologic and immunohistochemical features of these hybrid tumors suggest that the granular cells present have a common origin, most probably the odontogenic epithelium.
Seventeen cases of desmoplastic ameloblastoma were examined immunohistochemically. Immunoperoxidase techniques were applied for detection of keratin, desmin, vimentin and S-100 protein expression in these tumors. The tumor epithelium of desmoplastic ameloblastoma exhibited weak, focal, inconstant keratin staining, weak, variable expression of S-100 protein, desmin immunoreactivity of mild to moderate intensity and vimentin non-reactivity. The pertinent literature on the immunohistochemistry of ameloblastomas is briefly reviewed.
A five-month-old male baby presented with an abdominal mass which was found on computerised tomography (CT) to be involving the left kidney. Nephrectomy and histopathological study showed morphological featues of a malignant rhabdoid tumour. The tumour cells stained strongly for cytokeratin and epithelial membrane antigen and less intensely for vimentin. Electron microscopy revealed concentric whorled arrays of intermediate filaments within the tumour cell cytoplasm. The child was put on post-operative chemotherapy and radiotherapy but developed bilateral lung metastases and died three months after surgery.