Vol 46, No 4 (2018)


Current approaches to the morphological diagnosis of pancreatic neuroendocrine tumors and prediction of their clinical course based on the analysis of our own database

Gurevich L.E., Kazantseva I.A.


Aim: Combined clinical and morphological analysis of the pancreatic neuroendocrine tumor (pNET) spectrum according to the new World Health Organization classification: patient distribution, hormonal status, morphological grading, somatostatin receptor 2 (SSR2) and 5 (SSR5) expression, the choice of tissue-specific markers for the differential diagnosis of primary NET in the pancreas based on metastases with unknown primary tumor.

Materials and methods: The study was performed with 472 tissue samples from pNETs taken from patients. Morphological analysis consisted of histological and immunohistochemical examination with a panel of antibodies to chromogranin A, synaptophysin, CD56, insulin, glucagon, somatostatin, gastrin, calcitonin, adrenocorticotropic hormone (ACTH), serotonin, pancreatic polypeptide, cytokeratins (CK) of a wide spectrum, CK7 and CK19, p53, Ki-67, SSR 2 and SSR5, PDX-1, Isl-1, and NESP-55.

Results: In women, the prevalence of pNETS was 2.3 higher than in men (2.3:1). We were able to identify 299 (63.3%) insulinomas, 134 (28.4%) non-functioning NETs, 28 (5.9%) gastrinomas and 1.8% rare tumors (somatostatinomas, “calcitoninomas” and ACTH-producing). Metastatic tumors were found in 16.5% of the cases. Multiple endocrine neoplasia syndrome type 1 was confirmed in 11.9% of the pNET patients, and in 30.8% of those aged below 30 years. Multiple tumors (2 to 10) were found in 32 patients by the time of the diagnosis or occurred at 7 to 18 years after initial surgery. 28.3% of the tumors were CK19-positive, with 54.4% of them being metastatic. Insulinomas were least prone to metastasizing (5.7% of the cases), with 41.2% of them being CK19-positive. Metastases were found in 70.4, 66.7, 100, and 100% of gastrinomas, “calcitoninomas”, ACTH-producing, and somatostatinomas, respectively, with CK19-positivity found in 85.2, 66.7, 66.7, and 100% of these tumors. SSR2 expression was observed in all gastrinomas and “calcitoninomas”, in 90.5% of “glucagonomas”, 85.7% of PPomas, and 66.7% of somatostatinomas. SSR5 expression was significantly less frequent. 86.3% of the studied tumors were PDX-1-positive: all somatostatinomas, 97.4% of insulinomas, 92.3% of gastrinomas, 83.3% of PPomas, 80% of the non-functioning NETs. PDX-1-negativity was identified in all “calcitoninomas” and in 57.1% of the non-functioning “glucagonomas”. 83.3% and 90.9% of the pNETs were Isl-1 and NESP-55-positive, respectively.

Conclusion: Combined morphological and immunohistochemical examination of pNETs allows for the correct diagnosis, assessment of their prognosis and choice of the most effective treatment. The malignancy grade of pNETs depends on the cell immunophenotype and is higher in the cases with co-expression of the markers of neuroendocrine and ductal differentiation (CK19), as well as with ectopic hormonal production.

Almanac of Clinical Medicine. 2018;46(4):298-313
pages 298-313 views

Expression of the phosphorylated variant of the AKT1-kinase (p-AKT1) in well-differentiated pancreatic neuroendocrine tumors: immunohistochemical evaluation

Delektorskaya V.V., Solov'eva O.N., Chemeris G.Y., Patyutko Y.I.


Background: Well-differentiated pancreatic neuroendocrine tumors (pNETs) represent a group of rare epithelial neoplasms with a highly variable clinical course. AKT1 is one of the most frequently activated protein kinases in pNETs, which promotes the tumor growth and is of interest as a prognostic factor and a target for new treatment approaches.

Aim: To study the expression of the phosphorylated variant of AKT1-kinase (p-AKT1) in primary pNETs and their liver metastases and to correlate the results with various clinical and pathological parameters and the disease prognosis.

Materials and methods: P-AKT1 expression was studied by the immunohistochemical analysis of the primary lesions and liver metastases in 52 pNETs patients.

Results: A high level of cytoplasmic and/or nuclear immunoreactivity was detected in 24/52 of the primary pNETs (46.2%) and in 16/27 of their liver metastases (59.3%). p-AKT1 expression was observed in 3 (21.4%) of NET grade (G) 1, in 14 (46.7%) of NET G2, and in 7 (87.5%) of NET G3. p-AKT1 expression was more frequently identified in pNET G3 category and increased during the tumor progression in metachronous liver metastases, as compared to the corresponding primary tumor. In addition, p-AKT1 positivity was significantly associated with an increase of grade from G1 to G3 (p = 0.004), the Ki-67 index (p = 0.029), the pTNM stage (p = 0.0008), perineural invasion (p = 0.031) and a decrease in disease-free survival (p = 0.05).

Conclusion: The results suggest that p-АКТ1 plays an important role in the pathogenesis of pNETs and may be an additional criterion for assessment of the prognosis and treatment effectiveness in this type of tumors.

Almanac of Clinical Medicine. 2018;46(4):314-322
pages 314-322 views

ELISA study of matrix metalloproteinases 2, 7, 9 and their type 2 tissue inhibitor in the tumors of gastric cancer patients: clinical and pathologic correlations

Gershtein E.S., Ivannikov A.A., Chang V.L., Ognerubov N.A., Davydov М.M., Stilidi I.S., Kushlinskii N.E.


Background: Over the last 10 years the incidence of gastric cancer has declined significantly. Nevertheless, it remains one of the most prevalent malignancies both in Russia and worldwide. Therefore, the problems of early diagnostics, prognosis and individualized treatment choice are still on the agenda. Much attention is paid to the evaluation of molecular biological characteristics of the tumor, as well as to the development of multiparametric prognostic systems for gastric cancer based on its identified characteristics. An important place among potential tumor biological markers belongs to matrix metalloproteinases (MMPs) involved into all the stages of tumor progression, first of all, into the regulation of invasion and metastasizing.

Aim: Comparative quantitative evaluation of some MMP family members (MMP-2, 7, and 9) and one of the tissue MMP inhibitors (TIMP-2) levels in the tumors and adjacent histologically unchanged mucosa in gastric cancer patients, the analysis of their associations with the main clinical and pathological features of the disease and its prognosis.

Materials and methods: Sixty six (66) primary gastric cancer patients (32 male and 34 female) aged 24 to 82 years (median, 61 year) were recruited into the study. Twenty two (22) patients were with stage I of the disease, 11 with stage II, 28 with stage III, and 5 with stage IV. The concentrations of the proteins studied were measured in the tumor and unchanged mucosa extracts by standard direct ELISA kits (Quantikine®, R&D Systems, USA).

Results: Tumor MMP-2, 7 and 9 levels were significantly increased, compared to those in the adjacent histologically unchanged mucosa, in 80, 70 and 72% of gastric cancer patients, respectively, while the increase of TIMP-2 level found in 61% of the tumors was not statistically significant. Tumor MMP-2 and TIMP-2 content was increasing significantly with higher T index – size and advancement of the primary tumor (p < 0.01 and p < 0.05 respectively). Tumor MMP-2 level was also increasing in parallel with the N index (regional lymph node involvement; p < 0.01); it was significantly higher in the patients with distant metastases than in those without them (p < 0.05). Tumor MMP-9 and MMP-7 concentrations were not significantly associated with the indices of the tumor progression. The patients were followed up for 1 to 85 months (median, 18.3 months). According to the univariate analysis, high (> 32.6 ng/mg protein) MMP-2 and low MMP-7 (< 1.1 ng/mg protein) levels in the gastric cancer tissue represent statistically significant unfavorable prognostic factors for overall survival. Increased TIMP-2 level is associated with a non-significant decrease in the overall survival (p > 0.05), whereas the MMP-9 level was unrelated to the gastric cancer prognosis. Only T index (p = 0.0034) and tumor MMP-7 content (p = 0.026) remained independent prognostic factors in the multivariate regression analysis.

Conclusion: The majority of gastric cancer patients demonstrate a significant increase in the expression of three MMP family members, i.e. gelatinases (MMP-2 and 9), and matrilysin (MMP-7), in the tumors, as compared to adjacent histologically unchanged mucosa. Only MMP-2 levels were associated with the disease progression, increasing with higher TNM system indices. High MMP-2 and low MMP-7 content in the gastric cancer tissue are significant unfavorable prognostic factors for the overall survival in the univariate analysis, but only MMP-7 has retained its independent prognostic value in the multivariate assessment.

Almanac of Clinical Medicine. 2018;46(4):323-329
pages 323-329 views

Clinical and morphological characteristics of type 1 and 2 autoimmune pancreatitis

Lishchuk S.V., Dubova Е.A., Pavlov K.А., Udalov Y.D.


Rationale: In the recent years, an increased interest to autoimmune pancreatitis (AIP) has been seen, related to growing diagnostic potential. In its turn, this leads to an increase in numbers of diagnosed AIP cases. At present, two types of AIP have been described with diverse clinical manifestation and morphology of the pancreas. However, the reproducibility of the differential diagnosis between AIP type 1 and 2 is low even among pancreatic pathologists.

Aim: To identify criteria for the morphologic diagnosis of AIP type 1 and 2.

Materials and methods: A morphological study of biopsy and surgical specimens from 26 patients with AIP was performed. There were 22 cases of AIP type 1 and 4 cases of AIP type 2. In addition to hematoxylin eosin staining of the specimens, immunohistochemistry was used with counting of CD138+ absolute numbers, determination of IgG+ and IgG4+ cells in the inflammatory infiltrates, as well as the ratios of IgG4+/IgG+ and IgG4+/CD138+ cells.

Results: AIP type 1 was characterized by storiform fibrosis of the pancreatic tissue (81.8% cases), involving the parapancreatic fat tissue, by moderateto-severe lymphoplasmocytic infiltration and signs of obliterative/non-obliterative phlebitis. Type 2 AIP was characterized by severe fibrosis with predominantly periductal (centrilobular) fibrosis and mild chronic inflammatory infiltration of the pancreas, while there was no extension of fibrosis and inflammatory infiltration to the parapancreatic tissues in any case. The mean number of CD138+ cell in AIP type 1 was 101.2 ± 27.9 per 1 high-power field (HPF), and in AIP type 2, it was 42.8 ± 20.9 per 1 HPF. The mean absolute number of IgG+ cells in AIP type 1 was 99.6 ± 25.7 per 1 HPF, whereas in AIP type 2, 42.1 ± 20.8 per 1 HPF. In AIP type 1, the mean number of IgG4+ plasmatic cells in the infiltrates was 74.5 ± 27.2 per 1 HPF, whereas in AIP type 2, it was 3.4 ± 2.7 per 1 HPF. The IgG4+/IgG+ ratio was 75 ± 12.6% vs. 8.4 ± 6.2%, and the IgG4+/CD138+ ratio was 72.4 ± 12.3% vs. 8.3 ± 5.9% in AIP type 1 and type 2, respectively.

Conclusion: For the differential diagnosis of type 1 and 2 AIP, it is necessary to take into consideration not only typical histological abnormalities, but also the numbers of CD138+, IgG+ and IgG4+ cells within the inflammatory infiltrate, as well as the IgG4+/IgG+ and IgG4+/CD138+ ratios.

Almanac of Clinical Medicine. 2018;46(4):330-337
pages 330-337 views

Long-term survival of uveal melanoma patients after enucleation, depending on molecular genetic aberrations

Neroev V.V., Saakyan S.V., Amiryan A.G., Tsygankov A.Y., Burdennyy A.M., Loginov V.I., Khlgatyan M.R.


Rationale: In the recent years molecular genetic prognostic factors are becoming very important for predicting the course of uveal melanoma (UM). In clinical practice, molecular genetic methods are used to identify patients with a high risk of metastases.

Aim: To determine the survival of UM patients after enucleation, depending on molecular genetic aberrations.

Materials and methods: Thirty (30) patients with UM aged from 23 to 83 years were examined and treated. In all cases, enucleation was performed. The removed eyes underwent morphological and molecular genetic and cytogenetic analysis (loss of heterozygocity on chromosomes 1, 3 and 8, methylation of the RASSF1A gene, mutations in GNAQ/11 genes, polymorphism of the ABCB1 gene). The median follow-up was 61 months.

Results: The cumulative 3-year survival of the UM patients was 77.8 ± 8.0%, and the 5-year survival 63.0 ± 9.0%. The mean survival time was 52.8 ± 3.9 months. The patients with chromosome 3 monosomy showed significantly lower 5-year survival rates than the patients with partial monosomy and without loss of heterozygocity in chromosome 3 (log-rank test, χ2 = 14.111, p = 0.001). The loss of heterozygocity on chromosomes 1 and 8, the methylation of the RASSF1A gene, the mutations in GNAQ/11 genes, and the polymorphism of the ABCB1 gene were not associated with poorer vital prognosis.

Conclusion: Molecular genetic aberrations play an important role in predicting the course of the tumor process and determining the risk of hematogenous metastasizing in UM patients. The significant role of chromosome 3 monosomy has been proved. Due to the relatively small cohort (30 patients) and the time factor (analysis of 5-year survival), the role of other molecular genetic changes has not been confirmed, which requires an assessment of not only genetic, but also clinical, echographic and morphological prognostic factors.

Almanac of Clinical Medicine. 2018;46(4):338-346
pages 338-346 views

Clinical and morphological features of the leukoplakia in the Perm region

Sedova T.G., Khlebnikova A.N.


Leukoplakia is a chronic precancerous skin and mucosal lesion with a multifactorial etiology.

Aim: To retrospective analyze clinical and morphological characteristics of leukoplakia in the Perm region.

Materials and methods: The study was based on the data extracted from outand in-patient medical files and biopsy samples of 332 patients referred to the Perm Regional Oncological Dispensary (Perm, Russian Federation) in 2005 to 2016. The analysis of clinical and morphological characteristics was carried out with consideration of anatomical localization, gender and histopathological findings.

Results: Among the patients with leukoplakia, there were mostly women (77.7%) of the older age (61.6 ± 3.5 years), with frequent lesions of the vulvar mucosa (71.7%). Men had similar prevalence of genital (47.3%) and oral (52.7%) leukoplakia. Simple (flat) leukoplakia was found in 31.7% of the cases, verrucous in 45.0%, and erosive/ ulcerative in 23.3%. Verrucous leukoplakia was more often characterized by hyperkeratosis (95.5%) and vacuolar degeneration (94.8%). The erosive/ulcerative type was characterized by parakeratosis (95.7%), acanthosis (84.3%), dyskeratosis (90.0%), and dermal vascularization (95.7%). Dysplasia was observed in 52.3% of the cases, with 90% in erosive/ulcerative leukoplakia, 31.5% in simple leukoplakia and 47.4% in verrucous type. The frequency of dysplasia signs increased with an increase of the dysplasia grade (SIN).

Conclusion: In the Perm region, leukoplakia was more prevalent in female patients and had predominantly genital localization (96.9%). The risk of verrucous and erosive/ulcerative genital leukoplakia in women was 2,27and 1,75-fold higher than in men, respectively. In all clinical types, there was a slight predominance of SIN1, whereas the ratio of dysplasia grades was similar. Only combined assessment of clinical and morphological data could improve prediction of transformation to cancer.
Almanac of Clinical Medicine. 2018;46(4):347-354
pages 347-354 views

Gender particulars of morphological phenotypes of the right atrial appendage myocardium in patients with chronic heart failure who underwent an open heart surgery

Liskovа Y.V., Stadnikov A.A., Salikova S.P.


Background: For many decades, women in all age groups have a high risk of mortality and perioperative complications of cardiac surgery (CS), with its cause being unclear until now. Preoperative prediction of cardiovascular complications (CVC), based only on the clinical and instrumental criteria without taking gender into account, also remains unsuccessful. There is an opinion that the structural and functional changes in the myocardium, which exist before the operation, could significantly affect the patient's condition after cardiac surgery.

Aim: To identify morphological and molecular predictors of unfavorable prognosis in chronic heart failure (CHF) patients of both genders after CS.

Materials and methods: The study included 87 CHF patients of both genders referred for an elective cardiac surgery. Before the operation, a standard examination and treatment of CHF and concomitant disorders were performed. A sample of the right atrial appendage (RAA) myocardium that had been resected during the CS, was studied by histological, immunohistochemical (IHC) (expression of caspase-3, bcl-2, MMP-2, TIMP-1, p38α, CD-34) and morphometric methods. At days 10 to 14 after CS, the presence of CVC was assessed in all the participants. We examined the relationship between the development of CVC and morphological changes in the RAA myocardium before surgery, taking into account the patients' gender.

Results: Compared to the group with the favorable postoperative course in the myocardium, in the male/female group with unfavorable post-CS course there was a significant reduction in the cardiomyocyte (CMC) diameter (13.26 ± 3.14; p < 0.01 / 13.99 ± 3.64; p < 0.01), the bulk density (BD) of CMC (55.4 ± 9.45; p < 0.01 / 51.22 ± 10.12; p < 0.01) vol. %, a trophic index (0.24 ± 0.1; p < 0.01 / 0.21 ± 0.06; p < 0.01), as well as a significant increase in the stromal BD (44.91 ± 9.23; p < 0.01 / 47.78 ± 10.12; p <0.01) vol. % and the Kernogan index (1.78 ± 0.49; p < 0.01 / 1.43 ± 0.64; p = 0.143). IHC analysis of the RAA myocardium in the male/female group with an unfavorable postoperative course showed an increase in the amount of caspase-3 (+) CMC (3.9 ± 0.46; p < 0.01 / 3.34 ± 0.4; p < 0.01), an increase in the activity of +/++/+++ p38α (3/30/69; p < 0.01 / 2/39/60; p < 0.01) %, the expression of MMP-2 (2/56/43; p < 0.01 / 0/68/31; p < 0.01) %, with a decrease in the expression of TIMP-1 (19/29/52; p < 0.01 / 8/24/67; p < 0.01) % and BD of CD-34 stromal cells (18.46 ± 8.5; p < 0.01 / 27.54 ± 5.88; p < 0.01) %, compared with groups with a favorable current.

Сonclusion: The study showed the role of caspase-3, MMP-2, and CD-34 in the RAA myocardium as prognostic markers of CVC in the early postoperative period, as well as gender differences in modulation of the apoptotic pathways and inefficiency of anti-apoptotic mechanisms in the RAA myocardium. Based on the assessment of the RAA myocardial reorganization, an integral prognostic picture of the structural and functional changes in the myocardium has been proposed, which makes it possible to identify a special patient cohort with an exceptionally high risk of unfavorable course of the post-CS period.

Almanac of Clinical Medicine. 2018;46(4):355-366
pages 355-366 views


Rare clinical cases of “combined” skin carcinomas in cadaver renal allograft recipients

Kazantseva I.A., Gurevich L.E., Bobrov M.А.


The most prevalent skin cancer in the transplant organ recipients is squamous cell cancer, followed by basal cell cancer. The skin cancer incidence and related mortality in the transplant organ recipient are significantly higher than those in the general population, which is to be linked with prolonged pharmaceutical immunosuppression. Multiple tumors are also typical for this patient group. The article describes two rare combinations of skin carcinomas with different histological characteristics in patients with cadaver renal allograft (CRA). Clinical case 1: A 43-year old female patient. In 2011, she was transplanted with a CRA due to end-stage renal failure caused by congenital cystic dysplasia (multicystic kidney disease), with subsequent removal of the allograft at 1.5 months after the transplantation. In 2014, she had her second CRA transplanted and until now is on immunosuppressive therapy. In 2013, the patient noticed two slowly growing masses in her right and left supraclavicular areas; they were clinically assessed as basal cell cancer and surgically resected. Histological examination of the resected skin fragments showed squamous cell carcinoma focuses in situ (Bowen's disease), with alternating superficial basalioma focuses; proliferating keratinizing squamous cell cancer with polymorphous structure, with prevailing acantholytic type, morphoeic basal cell carcinoma and basal squamous cell carcinoma were found throughout the dermal layer, up to the subcutaneous tissue. Clinical case 2: A 63-year old male patient was transplanted with CRA in 2007, due to end-stage renal failure caused by nephrolithiasis and chronic pyelonephritis. During the examination performed in 2013, a mushroom-like tumor (with a 3 cm diameter and a stipe of 1 cm in diameter, with erythematous tuberous surface) was found on the anterior neck surface, near the suprasternal notch. The tumor was surgically resected within the normal skin. Histological and immunochemical examinations showed that the mass consisted of two different tumors closely adjacent one to the other and separated by a narrow dermal layer, namely, neuroendocrine Merkel cell skin carcinoma and porocarcinoma with some signs of squamous cell and sebaceous cell differentiation.

Conclusion: The descriptions of the rare cases confirm that transplantation-related skin cancers are highly relevant. Due to continuous renewal of the tissue components that is intrinsic to this organ, and due to deterioration of the immune control over proliferation and differentiation, they are characterized by a multiplicity of histological types and an unfavorable prognosis.

Almanac of Clinical Medicine. 2018;46(4):367-373
pages 367-373 views

Wollfian tumor: a case report and a literature review

Dubova E.A., Pavlov К.A., Lishchuk S.V., Tertychnyi A.S., Bakhvalova A.A., Bryunin D.V.


Wolffian tumor is a rare neoplasm arising from mesonephric duct remnants. Not more than 100 cases of the disease have been described. The authors present their own clinical observation of the Wolffian tumor in a 43-year old female patient. Radiological examinations resulted in the suspicion of a subserous leiomyoma of the uterus; however, the surgery showed a mass arising from the fallopian tube wall. Histologically, there was a growing tumor within the fallopian tube wall with no mucosal lesions. The tumor consisted of myxomatous stroma with cribriform, solid and reticular areas composed of relatively monomorphic, mildly eosinophilic cells with monotonous nuclei and single mitoses. On immunohistochemistry, the tumor cells expressed vimentin, pan-cytokeratin, cytokeratin 7 and calretinin. This case illustrates that final verification and differential diagnosis of the tumor with other fallopian tube neoplasms, including malignancies, is only possible with an immunohistochemical study.

Almanac of Clinical Medicine. 2018;46(4):374-378
pages 374-378 views

Clinical observation of metastatic thyroid disease in a patient with breast cancer

Bondarenko E.V., Gurevich L.E., Kotova I.V.


Metastatic tumors of the thyroid gland (TG) are rare. Usually thyroid gland metastases originate from renal, lung, skin and gastrointestinal cancers. Breast cancer metastases are more rare and in various samples amount to 3 to 34% of all cases of the metastatic thyroid disease. We present a rare case of metastatic carcinomatosis into the thyroid goiter in a 63-year old female patient who has received combination therapy for breast cancer. In 2016, right-sided mastectomy was performed due to the right breast cancer (invasive carcinoma, non-specific type, Grade 2, with skin invasion) with subsequent four chemotherapy courses. A slowly growing nodule in the thyroid gland was first found in 2012; in 2017, fine needle aspiration biopsy was performed, which showed a follicular tumor that resulted in thyroidectomy. At the histological examination, against the background of thyroid goiter, multiple small lesions with advanced nuclear polymorphism were found, with doubtful diagnosis. To clarify the histogenesis of the tumor lesions, immunohistochemical assessment was performed. Its first phase included the markers of primary thyroid tumors (thyroglobulin, TTF-1), and the second one consisted of the breast cancer diagnostic panel (mammoglobin, GATA-3, estrogen and progesterone receptors). The results showed multiple small metastases of the invasive breast carcinoma of non-specific type into the thyroid goiter. Taking into account eventual problems of differential diagnosis and significant morphological polymorphism of thyroid tumors, we recommend extending of the immunohistochemistry panel in this patient category.

Almanac of Clinical Medicine. 2018;46(4):379-383
pages 379-383 views

Choroidal melanoma, simulating an optic nerve tumor: a clinical case

Amiryan A.G., Panteleeva O.G., Maybogin A.M., Tsygankov A.Y., Saakyan S.V.


Choroidal melanoma is a primary malignant intraocular tumor with unfavorable vital prognosis. Factors predisposing to invasion of choroidal melanoma into the optic nerve include its juxtapapillary localization, the large tumor size, its diffuse growth, the presence of necrosis in the tumor, increased intraocular pressure and the epithelioid cell type of the tumor. The authors describe the clinical case of a 31-year-old patient with a primary diagnosis of an optic nerve neoplasm. Peripapillary edema and indistinct optic disc margins were found by ophthalmoscopy. There was a slightly protruding gray-slate lesion with indistinct boundaries near the optic nerve disc. The retina was edematous with multiple merging hemorrhages. The data obtained by echography (the focus protruding both inside the eye and along the optic nerve trunk), color Doppler mapping (single own vessels in the projection of the orbital part of the mass) and computed tomography allowed for the suspicion of the primary choroidal melanoma. To clarify the nature of the process, a trans-conjunctival orbitotomy with a revision of the orbit and the optic nerve was performed. The expansion of the proximal part of the optic nerve with partial breakthrough of the optic nerve shells was revealed intraoperatively. The patient underwent enucleation with morphological and molecular genetic verification of the primary choroidal melanoma with invasion of the optic nerve. The data obtained (absence of chromosome 3 monosomy, deletion of the short arm of chromosome 1, methylation of promoter regions of the RASSF1A gene, mutations in the 4 and 5 exons of the GNAQ gene, CT genotype of the polymorphic marker C3435T of the ABCB1 gene) indicate a relatively favorable vital prognosis, despite the extrascleral growth of the tumor and its mixed cell type. The patient underwent a course of anti-relapse proton irradiation of the orbit with a total dose of 58.8 Gy at 80–90% isodose. Currently, 7 years after the treatment, the patient continues to be followed up by ophthalmologists and oncologists, without any signs of local recurrence and distant metastases. This clinical example demonstrates the possibility of extraocular extension of a small intraocular choroidal melanoma simulating an optic nerve tumor, and confirms the need for a thorough clinical and instrumental examination for a correct diagnosis and adequate management of the patient.
Almanac of Clinical Medicine. 2018;46(4):384-389
pages 384-389 views

Kaposi’s sarcoma with the eyelid involvement (6 clinical cases)

Grishina E.E.


Kaposi's sarcoma is a multifocal tumor from vascular endothelium with a low grade of malignancy. It develops due to underlying immune deficiency and is associated with human herpesvirus 8. Kaposi's sarcoma of the eyelids is rare, and its diagnosis can be difficult both for ophthalmologists and oncodermatologists. The paper describes six clinical cases of Kaposi's sarcoma with involvement of the eyelids. Three patients had an HIV-associated type of the tumor. One patient had an immunosuppressive type of the tumor during immunosuppressive treatment after kidney transplantation. Two elderly patients had Kaposi's sarcoma of the classic type. Tumors of the eyelids developed after several years of skin involvement. All patients had advanced (nodular) stage of Kaposi's sarcoma of the eyelids, whereas the skin tumors looked as spots (maculas) or papules (macular or papular stage of the disease). The eyelid tumor presented as an extensive dark red tumor nodule distinctly separate from the adjacent tissues. In all cases, the eyelid tumor was big and hindered the sight. All the patients were treated by an oncodermatologist and/or a specialist in infectious diseases, depending on the clinical type of the disease. Kaposi's sarcoma rarely involves the eyelid skin or conjunctiva; however, in immunodeficient patients it must be included into the list for the differential diagnosis of eyelid tumors.

Almanac of Clinical Medicine. 2018;46(4):390-394
pages 390-394 views

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