Turkish Journal of Pathology - 2018; 34(3)

Evaluation of the Prognostic Importance of c-Myc and Bcl-2 Expressions and the Presence of Epstein-Barr Virus in Classical Hodgkin Lymphoma

2018-09-01

Objective: Although classical Hodgkin lymphoma (cHL) has a relatively good prognosis, it also entails different treatment responses and involves patients who have different clinical courses. Our aim was to investigate c-Myc, Bcl-2 and EBV biomarkers in cHL and their relationship with the IPS score. Material and Method: c-Myc and Bcl-2 immunohistochemical staining with EBER in situ hybridization (EBER-ISH) was applied to the paraffin sections of 94 cases diagnosed as cHL. These cases were classified into two groups as low and high clinical symptoms according to the International Prognostic Scores (IPS). Results: Positive results were obtained in 83 (88.3%) cases with c-Myc and 39 (43.5%) cases with Bcl-2 while EBER-ISH was found positive in 42 (44.7%) cases. No difference was found between the groups of low/high IP scores with respect to the positive or negative results of EBER-ISH, Bcl-2 and c-Myc. When Bcl-2 and c-Myc positive cases were grouped together and compared to the IP scores of the remaining cHL cases, again no difference was seen. Extranodal involvement and bone marrow involvement was observed in 25 (26.5%) and 9 (9.5%) cases, respectively. Similarly, no statistically significant differences was found between these groups according to their positivity with EBER-ISH, Bcl-2 and c-Myc. Conclusion: We could not find any relationship between Bcl-2, c-Myc and EBER-ISH positivity and the low/high IPS groups in cHL. New studies with larger series are needed in which more precise cut-off values are used and clinically and biologically heterogeneous groups of cHL patients are determined more clearly.

Gastrointestinal Stromal Tumors: A Clinicopathological and Immunohistochemical Study of 65 Cases

Merih TEPEO�LU, Gonca �ZG�N, M. Zeyneb TUNCA, Tugan TEZCANER, B. Handan �ZDEMIR — 2018-09-01

Objective: The clinical behavior of gastrointestinal stromal tumors is divergent. The aim of the present study was to define the clinicopathological features that determine the patient�s outcome. Material and Method: Sixty-five gastrointestinal stromal tumors were reviewed with their histological, immunohistochemical and clinical features and compared with their clinical outcome statistically. Results: Tumors were located in the stomach (n=39, 60%), small intestine (n=22, 33.8%) and large intestine (n=4, 6.2%). Immunohistochemically, CD 117 positivity was found in 90.8%, whereas CD34, Smooth muscle actin, Desmin and S100 positivity was found in 73.3%, 61.7%, 11.7% and 28.3% of tumors respectively. All six ��CD 117-negative�� cases expressed DOG-1. The mean Ki-67 proliferation index was 8.69%�12.76. Liver metastasis was detected in seven cases. A significant association was detected between decreased mean survival time and increased tumor size (p<0.001), large bowel localization (p=0.047), mitosis (p<0.001), the presence of necrosis (p=0.001), metastasis (p=0.033), Ki-67 proliferation index (p=0.002) and risk category (p<0.001). CD 34 positivity was mostly seen in the stomach (p=0.001), and CD 34 positive tumors had longer overall survival (92.85.�5.77 months versus 67.21�13.68 months) (p=0.046). Higher Ki-67 proliferation index (≥6%) was also correlated with the presence of metastases (p=0.015). Conclusion: Our study indicates that in addition to well-known risk factors such as increased tumor size, high mitotic activity and metastasis; higher Ki-67 proliferation index, the presence of necrosis, and CD34 negativity also correlate with shorter survival time.

Granulomatous Lobular Mastitis: Clinicopathologic Presentation of 90 Cases

Mine �Z�EN , �ahsine TOLUNAY , M. �ehsuvar G�KG�Z — 2018-09-01

Objective: Granulomatous lobular mastitis is a rare benign chronic breast disease first described by Milward in 1970, and then by Kessler and Wolloch in 1972. In this study, we aimed to present clinicopathologic features of granulomatous lobular mastitis with literature data. Material and Method: In this study, the archives of Uludag University Medical Faculty Department of Pathology were screened for granulomatous lobular mastitis cases between 2005 and 2017. Results: A total of 90 patients with granulomatous lobular mastitis diagnosed between 2005 and 2017 were identified. All of the cases were female. The mean age was 34+8.3 (range 21-60 years). There was sarcoidosis in one case and tuberculosis in another case, but no systemic disease was found in the charts of the other cases. Histopathological evaluation of 90 cases revealed non-necrotizing granulomas involving lobule-restricted, epithelioid histiocytes and Langhans-type multinuclear giant cells. There was no case of necrosis, including our only case with a history of tuberculosis. Conclusion: We conclude that our granulomatous lobular mastitis cases have similar characteristics with the series reported earlier, when all features are taken into consideration.

Fascin Expression in Ameloblastoma, Odontogenic Keratocyst and Dentigerous Cyst

2018-09-01

Objective: The purpose of this study was to assess and compare fascin expression in 4 lesions which differ in aggressiveness: odontogenic keratocyst, dentigerous cyst and two types of ameloblastoma (solid and unicystic), and to find out whether fascin expression is associated with aggressiveness of these lesions or not. Material and Method: Nine solid ameloblastomas , 12 unicystic ameloblastomas, 13 odontogenic keratocyst and 12 dentigerous cyst were assessed in this study. The slides were examined at x400 magnification. Finally the lesions were divided into two groups based on microscopic examination, �low expression� and �high expression�. Results: There were no significant differences between the lesions, except that fascin expression was slightly higher in unicystic ameloblastomas in comparison to other groups in intensity and count of the immunostaining cells. Conclusion: The results of this study suggest that local aggressiveness does not result in fascin expression. We suggest more studies with more samples, assessing expression of different proteins be done in the future.

Recurrence in Uterine Tumors with Ovarian Sex-Cord Tumor Resemblance: A Case Report and Systematic Review

2018-09-01

Objective: The aim of this study was to evaluate the prognostic factors of recurrence in uterine tumors resembling ovarian sex-cord tumors (UTROSCT) and to determine clinical-pathological characteristics, treatment options and outcome. Material and Method: An electronic literature search was conducted from 1976 to 2018. After the comprehensive evaluation and conjunction with our case, the study included 79 cases. Results: The median age at initial diagnosis was 49 years (range; 16-86 years). The age was under 40 years in 21 (26.6%) patients. Whereas 68 patients underwent at least hysterectomy, 9 patients had organ sparing surgery. There was necrosis in 4 (5.1%) patients, atypia in 16 (20.3%) patients, and infiltrative tumor border in 34 (43%) patients. At least one mitosis per 10 high power fields was determined in 36 (45.5%) patients. The tumor involved at least part of the myometrium in 54 (68.3%) patients. Median follow-up time was 30 months (range; 3-296 months). Recurrence was determined in 5 (6.3%) patients. The disease free survival (DFS) was significantly related only to surgery type. None of the pathologic features were associated with DFS. The 5-year DFS was 86% and 96% in patients who underwent organ sparing surgery or not, respectively (p=0.038). Conclusion: The accurate pathologic diagnosis of UTROSCT has great value in shaping surgical management and management during the follow-up period. Organ sparing surgery was related to poor DFS. Although recurrence is rare, it should be kept in mind for patients with UTROSCT.

Fibrous Dysplasia: Clinicopathologic Presentation of 36 Cases

Mine �Z�EN, �lviye YAL�INKAYA, Muhammed Sad�k BILGEN, Zeynep YAZICI — 2018-09-01

Objective: Fibrous dysplasia is a slowly progressing bone lesion resulting from displacement of the normal medullary bone with abnormal fibroosseous tissue. The aim of this study was to assess the similarities and differences of our cases in relation to published reports. Material and Method: In this study, the archives of the Uludag University Medical Faculty Department of Pathology were screened for fibrous dysplasia cases between 2004 and 2016. Results: Within the mentioned period, there were 36 cases diagnosed as fibrous dysplasia. There were 21 male, and 15 female cases with an average age of 27.8�14.8 years (range 7-79 years). The most frequently affected sites were femur, costae, and craniofacial bones. There was one case localized to metacarpal bone, a very rare affection site. There were 4 polyostotic cases including 2 cases of McCune-Albright syndrome. Pelvic bone was affected in the polyostotic type, similar to published reports. Unlike former reports, however, long tubular bones were affected in male patients in our series. In our series, 32 cases had classical fibrous dysplasia, 3 cases had fibrocartilaginous, and one case had fibroosseous variants. Four cases localized to costae were accompanied by aneurysmal bone cyst. The presenting symptom was pathological fractures in a total of 4 cases, 3 localized to the femur, and 1 to the costa. Recurrence occurred in 5 cases treated with curettage. Two of the monostotic fibrous dysplasia cases developed malignant transformation into osteosarcoma. Conclusion: We conclude that our series of fibrous dysplasia cases have slight differences and mainly similar characteristics with the series reported earlier, when all features are taken into consideration.

Evaluation of All Surgical Margins in Pancreatic Resection Specimens by Proper Grossing Techniques: Surgical Pathology Experience of 285 Cases

�zg�r EK�NC� — 2018-09-01

Objective: The aim of this study was to review our series of pancreatic resection specimen handling results and focus on the positivity of the tumor in various retroperitoneal surgical margins. Material and Method: Our archival cases from 2008 to 2018 were retrospectively examined, especially for the surgical margins. The demographics, tumor locations, and the diagnoses were recorded. The state of all of the retropancreatic surgical margins (anterior, posterior, superior, inferior, superior mesenteric vein and artery) were recorded. Results: There were 285 cases, of which 157 were male and 128 female. The mean and median ages were 63.3 and 64, respectively. Invasive ductal adenocarcinoma was the most common diagnosis [202 cases (70.8%)]. Positivity was observed in 90 (31.5%) margins. The majority was in the superior mesenteric vein margin [n:24 (8.4%)]. This was followed by the anterior, resection and SMA margins. Conclusion: Pancreatic resections should macroscopically be sampled by recommended methods in order to detect positivity in individual margins by proper grossing techniques. When this is applied, the superior mesenteric vein margin is the margin most prone to be positive for the tumor.

Desmoplastic Fibroblastoma in Maxillary Alveolar Bone Mimicking An Odontogenic Lesion: A Novel Case Report with Review of Literature

Zohreh JAAFARI-ASHKAVANDI, Mehdi YASAMIN SHIRAZI, Sepideh ASSAR — 2018-09-01

Desmoplastic fibroblastoma (Collagenous fibroma) is a rare benign soft tissue tumor that disseminates widely and can be intramuscular or subcutaneously. There are seven reported cases in the oral cavity so far. We reported a novel case of collagenous fibroma that has been found in the anterior part of maxilla in a 58-year-old woman as an intraosseous radiolucent lesion that has been discovered during routine radiography. Histopathologically, spindle and stellate-shaped fibroblasts in a collagen-rich stroma as well as binucleated and multinucleated fibroblasts with prominent nucleoli were seen. Desmoplastic fibroblastoma could be found in jaw bones and excision of the lesion is curative, and no aggressive growth has been reported.

Primary Paediatric Renal Primitive Neuroectodermal Tumor: A Case Report and Review of the Literature

2018-09-01

Ewing sarcoma/primitive neuroectodermal tumor (PNET) is a high-grade malignant neoplasm commonly affecting bones of the thoracic region. Primary Ewing sarcoma/PNET of the kidney is exceptional; it commonly affects young adults and is rarely reported in children. Here we describe a case of renal PNET in a 10-year-old girl who presented at our institute with haematuria and abdominal lump. Computer tomography scan revealed a huge mass in the right kidney. Computer tomography-guided aspiration from the mass showed cytomorphological features of a small blue round cell tumor. The patient underwent right radical nephrectomy. Histopathology with supplementary immunohistochemistry confirmed the diagnosis of PNET. She has been receiving treatment with adjuvant chemotherapy post-surgery and is currently disease free. Primary renal PNET is a distinct and rare entity. This tumor is very aggressive with low survival rate, even with a multi-disciplinary approach. We reported the case because it is rare in children, poses diagnostic challenges, is aggressive in behaviour and responds poorly to treatment.

Cloacogenic Adenocarcinoma of the Vulva: A Case Report and Review of the Literature

Merih TEPEO�LU, Halit �NER, A.Nihan HABERAL, �zlem �ZEN, Esra KU��U — 2018-09-01

Primary adenocarcinoma of the vulva, unrelated to the native glands of perineum is an extremely rare neoplasm. Despite awareness of this lesion for over 40 years, the origin is not beyond speculation. The most reasonable hypothesis is based on the remnants of cloacal differentiation during early days of life. Here we report the case of a 60-year-old patient with a vulvar mass, who underwent partial vulvectomy and bilateral regional lymph node dissection. The tumor was composed of papillary and complex glandular structures and exhibited diffuse positivity for cytokeratin 20 and polyclonal CEA, CDX2, and focal positivity with cytokeratin 7. Unlike the indolent behavior of this malignant neoplasm according to the literature, we found two metastatic inguinal lymph nodes. She did not receive adjuvant therapy and is still alive, free of disease 38 months after surgery. We present different aspects of vulvar adenocarcinomas with a case report.

Neu-Laxova Syndrome: An Unusual Association with Kyphosis

Amandeep KAUR, Vijayalaxmi SURANAG�, Kamal PAT�L, Hema BANNUR — 2018-09-01

The Neu-Laxova syndrome is a rare autosomal recessive condition associated with neuro-ectodermal abnormalities and other patterns of severe malformations leading to prenatal or early postnatal lethality. Association with kyphosis is an extremely rare finding. A fetus born from a 25-year-old gravida at 30 weeks gestation was diagnosed with Dandy Walker syndrome with severe intrauterine growth restriction on ultrasonography. On post-mortem examination after termination of pregnancy, the fetus showed facial dysmorphology with microcephaly, smooth shiny skin and kyphosis. Skin histology showed hyperkeratosis, edema and increased subcutaneous fat suggestive of ichthyosis. On the basis of gross and microscopic features seen, a diagnosis of Neu-Laxova syndrome was made.

Congenital Presentation of a Solitary Superficial Angiomyxoma in the Parotid Region Masquerading as Parotid Tumor

Shailaja SHUKLA, Shivali SEHGAL, Pawani PRABHAT, Khushboo DEWAN — 2018-09-01

Superficial angiomyxoma is a rare cutaneous and benign lesion. We present a case of congenital presentation of a superficial angiomyxoma in the parotid region in a 9-year- old female that was misdiagnosed as a parotid tumor. Appropriate diagnosis is important since such lesions have a good prognosis and rarely affect deeper structures. Possibility of superficial angiomyxoma should be kept in mind in the differential diagnosis of lesions of the parotid region.

A Case Report of Calcifying Pseudoneoplasm of the Neuraxis: An Incidental Finding After a Car Accident

2018-09-01

Calcifying pseudoneoplasms of the neuraxis are rare, poorly understood lesions that can occur anywhere in the central nervous system without any age or sex predilection. Their pathogenesis is still controversial. Patients can present with various symptoms due to lesion location and only few cases have been discovered incidentally. We present a 25-year-old male referred to our hospital after a car accident. Computed tomography scan and magnetic resonance imaging was done because of the patient's head trauma and a calcified intracranial lesion was found and excised surgically. Pathologic evaluation of the specimen was consistent with the diagnosis of calcifying pseudoneoplasm of the neuraxis.

Fibromuscular Dysplasia of the Coronary Arteries: A Case Report and Review of the Literature

Julia KUZYK, Oksana BOIKO, Taras STETSKO — 2018-09-01

Fibromuscular dysplasia is a nonatherosclerotic and non-inflammatory vascular disease with primary lesion of renal and internal carotid arteries. We present a neonatal case of fibromuscular dysplasia who died on the second day of life. The newborn suffered from fibromuscular dysplasia of the coronary arteries and a congenital heart defect. The interesting feature of this case was the formation of aneurysms of the coronary arteries with pulmonary atresia. This case demonstrates a casuistically rare form of association between fibromuscular dysplasia of the coronary arteries and pulmonary artery atresia.