scholarly journals The clinico-pathologic profile of primary and recurrent orbital/periorbital plexiform neurofibromas (OPPN)

PLoS ONE ◽  
2021 ◽  
Vol 16 (10) ◽  
pp. e0258802
Author(s):  
Mohammad Alabduljabbar ◽  
Diego Strianese ◽  
Osama Al-Sheikh ◽  
Hind M. Alkatan ◽  
Hailah Al-Hussain ◽  
...  
Keyword(s):  
Alcian Blue ◽  
Smooth Muscle Actin ◽  
Neurofibromatosis Type ◽  
Proliferation Index ◽  
Diffuse Type ◽  
Ki 67 ◽  
Primary Tumors ◽  
Plexiform Neurofibromas ◽  
Recurrent Tumors ◽  
S 100

To evaluate and compare the clinical and histopathological profile of primary and recurrent orbital-periorbital plexiform neurofibromas (OPPN) in patients with neurofibromatosis type 1. We retrospectively evaluated 43 primary or recurrent neurofibroma (NF) specimens from 26 patients (2002 to 2018) at the King Khaled Eye Specialist Hospital, Saudi Arabia. Demographics, clinical presentation, and surgical intervention data were collected. Histopathological specimens were studied with hematoxylin-eosin, Alcian blue, and immunohistochemical markers; S-100, CD44, CD117, smooth muscle actin (SMA), neurofilament, and Ki-67. Of the 43 NFs specimens, 20 were primary and 23 recurrent tumors. For primary NF, the ratio of plexiform to the diffuse type was 13:7, however in recurrent tumors was 3:8 after the first recurrence, and 1:5 after multiple recurrences. Of the 17 patients with primary tumors that had paired recurrent tumors, 12/17 (70.6%) primary NFs were plexiform and 5/17 (29.4%) were diffuse. However, when tumors recurred, 13/17 tumors (76.5%) were diffuse and only 4/17 tumors (23.5%) had a plexiform pattern. The odds of a tumor having a diffuse pattern in recurrent NF was significantly higher than the plexiform pattern [OR = 7.8 (95% confidence interval 1.69:36.1) P = 0.008]. Primary plexiform NFs underwent an excision at a significantly younger age than the diffuse type. Recurrent NFs had significantly higher CD44, CD117, and neurofilament labeling (P = 0.02, P = 0.01 and P<0.001 respectively) but had significantly decreased Alcian blue, and S-100 labeling (P = 0.03, and P = 0.02 respectively) compared to primary tumors. SMA and Ki-67 proliferation index were not different between primary and recurrent NFs (P = 0.86, and P = 0.3 respectively). There appears to be a high risk for primary plexiform NFs to develop a diffuse histologic pattern when they recur. Immunohistochemical staining suggests a role of mast cells (CD117) and expression of infiltration makers (CD44) in the transformation of plexiform tumors to the diffuse phenotype.

scholarly journals A Rare Primary Tumor of the Thyroid Gland: A New Case of Leiomyoma and Literature Review

2018 ◽  
Vol 12 ◽  
pp. 117955491881353
Author(s):  
Yanling Zhang ◽  
Heng Tang ◽  
Huaiyuan Hu ◽  
Xiang Yong
Keyword(s):  
Thyroid Gland ◽  
Tumor Cells ◽  
Immunohistochemical Staining ◽  
Smooth Muscle Actin ◽  
Frozen Sections ◽  
Ki 67 ◽  
Eosinophilic Cytoplasm ◽  
S 100 ◽  
Painless Mass ◽  
The Right

Primary leiomyomas of the thyroid are very rare. We here report a case of a 53-year-old woman with a painless mass at the right thyroid, revealed by physical examination. The patient underwent a lobectomy. Frozen sections showed a spindle cell tumor of the thyroid gland. The nuclei of some of the tumor cells were obviously enlarged and deeply stained. Pseudocapsule invasion was observed in small foci. Samples showed neither mitosis nor necrosis and the nature of the tumor was difficult to determine. Paraffin sections showed a well-circumscribed nodular composed of intersecting fascicles of spindled to slightly epithelioid cells with eosinophilic cytoplasm and blunt-ended, cigar-shaped nuclei. We observed no significant nuclear atypia, mitotsis, or necrosis. Immunohistochemical staining showed the tumor cells to be positive for α-smooth muscle actin and h-caldesmon but negative for TG, TTF1, PAX8, S-100, CT, CK, and CD34. The ki-67 index was very low (<1%). Primary thyroid leiomyoma is rare and difficult to diagnose using frozen sections. Diagnosis requires immunohistochemical staining. Leiomyoma may be mistaken for other thyroid tumors also characterized by spindle cells.

Relationship of Ki-67 proliferative index and metastatic tumor of breast cancer.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e22190-e22190
Author(s):  
Kokoro Kobayashi ◽  
Yoshinori Ito ◽  
Akiko Ogiya ◽  
Naoya Gomi ◽  
Rie Horii ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Primary Tumor ◽  
Metastatic Tumor ◽  
Labeling Index ◽  
Proliferation Index ◽  
Luminal Breast Cancer ◽  
Line Treatment ◽  
Metastatic Tumors ◽  
Ki 67 ◽  
Primary Tumors

e22190 Background: The metastatic breast tumor tends to be more aggressive with high proliferation, but this has not been proven in clinical sampling of metastatic tumors. Methods: Forty-eight patients who had histological specimens of both primary and metastatic sites of luminal breast cancer (ER and/or PgR positive and HER2 negative) were examined. We classified them as luminal A (LA) with Ki-67 labeling index of less than 14% and as luminal B (LB) with Ki-67 labeling index of more than 14%. We analyzed their overall survival (OS) and progression free survival (PFS) of 1st line treatment of each subtype of primary and metastatic tumors. Results: Subtypes of primary tumors and metastatic tumors were as follows; the primary tumor: LA; 34 patients (70.8%), LB; 14 (29.2%), metastatic tumors: LA; 21 (43.8%), LB; 27 (56.2%). Patients with LA of the primary tumor demonstrated statistically longer OS (LA; 72.5 months, LB 39.6 months, p=0.009). OS depended on the subtype of the primary tumor. In contrast, patients with LB of a metastatic tumor showed a statistically worse PFS (LA; 20.5 months, LB; 11.5 months, p=0.040). PFS of the 1st line treatment for MBC depended on the subtype of the metastatic tumor. Conclusions: The frequency of LB was increased on metastatic tumors and tended to acquire a higher proliferation index. This suggests that characterization of metastatic tumors could be better as an indicator of subsequent treatment for MBC. [Table: see text]

scholarly journals Alterations in Expression of Cell Surface and Cell Cycle Signaling Molecules in Recurrent Nonmuscle Invasive Bladder Cancers: A Tissue Microarray Expression Analysis

2018 ◽  
Vol 4 (Supplement 2) ◽  
pp. 94s-94s
Author(s):  
V. Agrawal ◽  
N. Bharti
Keyword(s):  
Cell Cycle ◽  
Cell Surface ◽  
Surface Proteins ◽  
Proliferation Index ◽  
Beta Catenin ◽  
Ki 67 ◽  
Time To Recurrence ◽  
Recurrent Tumors ◽  
The Mean ◽  
Cell Cycle Signaling

Background: Nonmuscle invasive bladder cancers (NMIBC) are one of the most common urological cancers having a high risk of recurrence and progression. Recurrent tumors may acquire certain molecular alterations responsible for progression of the tumors. Identification of these alterations is important to understand the pathobiology and guide further management. Aim: We studied the differences in expression of cell surface proteins and cell cycle signaling molecules in primary and recurrent NMIBC by immunohistochemistry (IHC) on tissue microarrays (TMA). Methods: Using FFPE tissue, TMA of 82 tumors (40 primary NMIBC and 47 recurrences) were constructed. IHC for growth factor receptors [epidermal growth factor receptor (EGFR), HER2/neu and FGFR3], cell adhesion molecules (E-cadherin and beta-catenin) and cell cycle pathway molecules (p53, p21/WAF1/Cip1 and Ki-67 proliferation index) was performed. A semiquantitative H-score (Histo-score; range 0-300) was calculated according to the intensity (0, negative; 1, weak; 2, moderate; and 3, strong) and percentage of cells stained. < 10% cells showing nuclear p21 expression was considered p21-loss. The differences in expression between the primary and recurrent tumors were analyzed using paired t test. Results: The mean age at presentation was 65.3 ± 13.6 years with a male predominance (n=36). The mean time to recurrence was 33.4 months (range 3-109). Progression in grade and/or stage was seen in 30 (75%) tumors. Time to recurrence was shorter in primary tumors with ≥ 5% Ki-67 proliferation index. There was no significant difference in expression of cell surface proteins between primary and recurrent tumors. Significant p21 loss was seen in recurrent tumors ( P = 0.03) and significantly correlated with loss of surface beta-catenin and nuclear p53 positivity ( P = 0.002). Ki-67 index was higher in recurrent tumors and also correlated with p53 positivity ( P = 0.007). Conclusion: We found no significant differences in expression of cell surface molecules in primary nonmuscle invasive bladder cancers and their recurrences. However, there were significant alterations in expression of molecules of cell cycle signaling pathway and cellular proliferation in recurrent tumors suggesting the role of cell cycle regulators as promising targets in these cancers.

scholarly journals A Huge Pelvic-Abdominal Malignant GIST Tumour in a Patient with Neurofibromatosis Type 1: Case Report and Literature Review

2020 ◽  
Vol 2020 ◽  
pp. 1-9
Author(s):  
Islam Omar ◽  
Hani Alsaati ◽  
Ejaz Waris
Keyword(s):  
Signs And Symptoms ◽  
Neurofibromatosis Type ◽  
Imatinib Treatment ◽  
Ki 67 ◽  
Common Location ◽  
Rare Tumours ◽  
Gastrointestinal Tumours ◽  
S 100 ◽  
Abdominal Compartment

Gastrointestinal stromal tumours are rare tumours of the gastrointestinal tract (GIT) accounting for 0.1%–3% of all gastrointestinal tumours. The most common location is the stomach (55%) followed by the small bowel (31.8%), colon (6%), other various locations (5.5%), and the oesophagus (0.7%). They may also occur in extraintestinal locations. The signs and symptoms of GIST depend on the tumour’s location and size. Gastrointestinal bleeding is one of the most common symptoms. Other signs and symptoms include abdominal discomfort, pain or distention; intestinal obstruction, and weight loss. The association between the development of GISTs and neurofibromatosis 1 (NF1) has been established. NF1-associated GISTs tend to have a distinct phenotype, and the absence of KIT/PDGRFα mutations in turn has implications on further management when they do not respond well to imatinib treatment. Here, we present one of the largest GISTs reported in the literature with a total volume of 25.3×20×14 cm+27.9×23×8 cm and an overall weight of 7.3 kg, which developed in a 43-year-old female patient with NF1 and was resected on an emergency basis due to the rapid deterioration and development of abdominal compartment syndrome. Pathology assessment showed a malignant GIST composed of spindle cells with elongated nuclei with necrosis, marked pleomorphism and numerous giant cell. The mitotic count was >15/50 HPF, Ki 67 was 80%, and the lymphovascular invasion was clear. Immunohistochemistry investigations showed that Vimentin, CD117, and DOG1 were positive, while BCL-2 and CD99 were focal positives. Pan-CK, S-100, CD34, Desmin, SMA, and HMB-45 were negatives.

scholarly journals DIFFICULTIES OF DIFFERENTIAL DIAGNOSIS BETWEEN ATYPICAL LEIOMYOMA AND LEIOMYOSARCOMA OF THE UTERINE BODY

2016 ◽  
Vol 7 (1) ◽  
pp. 70-74
Author(s):  
E A Kogan ◽  
M A Solomakhina ◽  
S I Askolskaya ◽  
Yu V Popov ◽  
A V Kozachenko ◽  
...  
Keyword(s):  
Smooth Muscle Actin ◽  
Molecular Genetic ◽  
Genetic Research ◽  
Progesterone Receptors ◽  
Proliferation Index ◽  
Ki 67 ◽  
Gynecological Diseases ◽  
Reproductive Aged Women ◽  
Leiomyoma Of The Uterus ◽  
Uterine Body

Leiomyoma of the uterus is a benign hormone-dependent tumors in women.The incidence of LM among gynecological diseases is 25 to 50%, according to different authors. In reproductive-aged women its frequency is about 30%. WHO recommends to allocate leiomyoma, which in russian literature often referred to as a simple or ordinary leiomyoma, and its histologic variants (cellular, mitotic activity, epithelioid, myxoid, atypical leiomyoma and lipoleyomioma etc.).We performed clinical and morphological analysis of atypical uterine leiomyoma in 44-year-old woman. Immunohistochemical study detected positive reaction to smooth muscle actin, dismin, progesterone receptors, N-caldesmon. Adverse reactions to c-kit, ER keratins. Proliferation index by Ki-67 does not exceed 10%. At the molecular genetic research in the studied loci, the microsatellite instability and loss of heterozygosity, characteristic of the LMS, were not revealed.

scholarly journals A Rare Case of Aggressive Systemic Mastocytosis Involving the Gastrointestinal System and Review of Literature

2021 ◽  
Author(s):  
Jianjun Wang ◽  
Zhong Zheng ◽  
Feng-nan Niu ◽  
Ting Wang ◽  
Biao Zhang ◽  
...  
Keyword(s):  
Mast Cells ◽  
Tumor Cells ◽  
Systemic Mastocytosis ◽  
Clinical Manifestations ◽  
Skin Lesions ◽  
Proliferation Index ◽  
Pelvic Cavity ◽  
Ki 67 ◽  
S 100 ◽  
Hepatic Portal

Abstract Background Systemic mastocytosis is a rare disease and most patients have pigmented urticarial skin lesions. It can be easily missed and misdiagnosed in small biopsies, especially in those patients with nonspecific clinical complaints or untypical skin lesions. Case presentation We report a case of 69-year-old man who have presented with 2-year of diarrhea, progressive weight loss of 20kg and abdominal distention for 3 months. Ultrasound and abdominal CT scan showed massive effusions in abdominal and pelvic cavity. Colonoscopy was performed and showed intensive mucosal proliferations forming polypoid appearances. Microscopically, monotonously uniform, small, round tumor cells with slightly rich cytoplasm were concentrated between the residual glands in the colonoscopic biopsy. The tumor cells showed positive expression of CD117 and S-100, and low Ki-67 proliferation index of 2%, while Trypsin, CK, CD68, CD1a, Langerin, Syn, CgA, CD56, SATB2, CD20, CD3, α-inhibin and SMA were all negative. KIT D816V mutation was detected as well. Liver biopsy showed that CD117 positive cells were more than 15 cells in aggregates around the hepatic portal area and less than 15% mast cells were found in bone marrow smears. No multiple or diffuse pattern of mast cells infiltration was seen by repeated skin biopsies of skin lesions. With all these considered, the diagnosis of aggressive systemic mastocytosis was made. Conclusions The diagnosis of aggressive systemic mastocytosis is challenging for untypical clinical manifestations and subtle or inconspicuous lesions, especially in endoscopic biopsies, which requires awareness and a close teamwork of pathologists and clinicians.

scholarly journals Metachronous double primary neuroendocrine tumors in larynx and lung: a case report

2020 ◽  
Vol 48 (11) ◽  
pp. 030006052096292
Author(s):  
In Hye Song ◽  
Youn Soo Lee ◽  
Dong-Il Sun ◽  
Yong-Kil Hong ◽  
Kyo-Young Lee
Keyword(s):  
Tumor Cells ◽  
Carcinoid Tumor ◽  
Neuroendocrine Tumors ◽  
Lower Lobe ◽  
Proliferation Index ◽  
Atypical Carcinoid ◽  
Voice Change ◽  
Large Tumor ◽  
Ki 67 ◽  
Primary Tumors

When a patient harbors two or more neuroendocrine tumors (NETs), it can be difficult to determine whether they are double primary tumors or metastases. A 60-year-old man complained of voice change lasting 1 month. On physical examination and imaging, a 1.8-cm mass was observed in his epiglottis, and a laser epiglottectomy was performed. Upon microscopic examination, the tumor consisted of medium-sized ovoid or short spindle cells. Immunohistochemical staining of the tumor cells was positive for synaptophysin, chromogranin, and calcitonin but negative for CD56; the Ki-67 proliferation index was approximately 5%. The patient was diagnosed with atypical carcinoid tumor. In 2015, a hypermetabolic endobronchial tumor was identified in the left lower lobe by positron emission tomography-computed tomography. Bronchoscopic biopsy revealed palisading large tumor cells with high nuclear-cytoplasmic ratio, frequent mitoses, and necrosis. The tumor cells were positive for CD56 and negative for cytokeratin-7, thyroid transcription factor-1, P40, synaptophysin, chromogranin, and calcitonin; the Ki-67 proliferation index was approximately 90%. Overall histologic findings were consistent with large cell neuroendocrine carcinoma rather than metastatic atypical carcinoid tumor. Detailed clinical and pathological review are essential to differentiate between metastatic NET and double primary NETs and, therefore, to provide the best management of the patient.

Unusual Case of Multiple Synchronous Gastrointestinal Stromal Tumors of the Jejunum Presenting as an Arteriovenous Malformation

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S56-S56
Author(s):  
A Murzabdillaeva ◽  
H Taha ◽  
R J Hausner
Keyword(s):  
Arteriovenous Malformation ◽  
Epigastric Pain ◽  
Surgical Excision ◽  
Neurofibromatosis Type ◽  
Proliferation Index ◽  
Double Balloon Enteroscopy ◽  
Ki 67 ◽  
Genetic Features ◽  
Carney’S Triad

Abstract Introduction/Objective Gastrointestinal stromal tumor (GIST) arises from the interstitial cell of Cajal. 30% of GISTs arise in the jejunum. GISTS are generally solitary although multiple GISTs may be synchronous or metachronous. There are a few reports of a GIST of the jejunum mimicking a vascular malformation/arteriovenous malformation (AVM) prior to surgical excision. Methods Herein, we report a case of four synchronous GISTs of jejunum. One GIST, highly vascularized, presented with lower gastrointestinal bleeding. The patient was a 40-year-old female presenting with epigastric pain and melena. Results She was found to have an ulcerated lesion in the distal jejunum by capsule enteroscopy and double balloon enteroscopy, interpreted as an arteriovenous malformation and tattooed. Several other non-ulcerated “polyps” were described. Gross examination of the ensuing segmental resection of jejunum demonstrated four bosselated tumors ranging from 3.1 cm to 6.5 cm. Microscopically, three tumors did not extend to the surface of the jejunum and were predominantly composed of spindle cells. One tumor (identified by tattoo) extended to the surface with ulceration. This tumor was dominated by wide vascular channels with a spindle cell component between the channels. The four tumors were each positive for CD117, DOG1, and Succinate dehydrogenase B (SDH), identifying the four tumors as non-SDH-deficient GISTs. Ki-67 proliferation index was less than 5% in all four masses. Conclusion Multiple GISTs are rare, classified as either sporadic or familial. Familial GEISTs are described in neurofibromatosis type 1, Carney’s triad and Carney-Stratakis syndrome. Pediatric GISTs, with clinical and genetic features often differing from typical adult tumors may also be multiple. Our patient’s four GISTs are considered multiple synchronous sporadic neoplasms. This case serves as an important reminder for pathologists consider GIST in the evaluation of a highly vascular gastrointestinal proliferation and keep in mind the possibility of multiplicity.

scholarly journals Laparoscopic Resection of Schwannoma of the Ascending Colon

2015 ◽  
Vol 9 (1) ◽  
pp. 15-19 ◽  
Author(s):  
Yoshihiko Tashiro ◽  
Fumio Matsumoto ◽  
Keiko Iwama ◽  
Ai Shimazu ◽  
Sei Matsumori ◽  
...  
Keyword(s):  
Submucosal Tumor ◽  
Smooth Muscle Actin ◽  
Immunohistochemical Analysis ◽  
Right Hemicolectomy ◽  
Ascending Colon ◽  
Ki 67 ◽  
D2 Lymph Node Dissection ◽  
Benign Schwannoma ◽  
S 100 ◽  
Laparoscopic Right Hemicolectomy

Schwannomas of the colon are rare and difficult to diagnose preoperatively. We report a case of schwannoma of the ascending colon that was resected laparoscopically. A 64-year-old woman was referred to our hospital by her local clinic for further evaluation and management of a submucosal tumor of the ascending colon. A definitive preoperative diagnosis could not be reached despite examinations. Gastrointestinal stromal tumor, leiomyoma and lymphoma were the differential diagnoses. We performed a laparoscopic right hemicolectomy with D2 lymph node dissection. Histological findings with hematoxylin-eosin staining revealed spindle-like tumor cells, and immunohistochemical analysis showed that the tumor was positive for S-100 but negative for c-kit, CD34, smooth muscle actin and desmin, with a Ki-67 index of <5%. Thus, the diagnosis in this case was benign schwannoma of the ascending colon.

scholarly journals Histopathological Features and Immunophenotype of Primary Gastric Invasive Fibromatosis

2020 ◽  
Author(s):  
Yangkun Wang ◽  
Zhishang Zhang ◽  
Bo Jiang ◽  
Chaoya Zhu ◽  
Sunan Wang ◽  
...  
Keyword(s):  
Hormone Receptors ◽  
Tumor Tissue ◽  
Gastric Wall ◽  
Clinical Manifestations ◽  
Nerve Tissue ◽  
Proliferation Index ◽  
Fatty Tissue ◽  
Ki 67 ◽  
Positive Expression ◽  
S 100

Abstract Background To investigate the histopathological characteristics, immunophenotype and differential diagnosis of primary gastric invasive fibromatosis.Methods The clinical manifestations, histological morphology and immunophenotype of 4 cases of primary gastric invasive fibromatosis were observed and related literatures were reviewed.Results Among the 4 patients, 2 were males and 2 were females, aged 28 and 47 years, respectively. The lesions were located in the stomach in 2 cases, the gastric antrum in 1 case and cardia in 1 case. Under the microscope, the tumor was located in the submucosa, growing infiltratingly, and infiltrating into the gastric wall muscularis, serous membrane and extraserous fatty tissue. Tumor cells were rich in cytoplasm, with unclear cell boundaries, long rod-shaped nuclei, deep chromatin, no atypia, and rare mitotic figures. The tumor tissue was composed of proliferating spindle-shaped fibroblasts and collagen fibers, and the cell morphology was relatively uniform, arranged in parallel bundles or staggered weaves. Tumor tissue invaded and destroyed smooth muscle, blood vessels, nerve tissue and adipose tissue of the gastric wall. Immunophenotype: positive expression of vimentin, β-catenin, SMA positive expression; CKpan, EMA, S-100 protein, desmin, CD99, Bcl-2, ALK, CD34, CD117, DOG1, hormone receptors were all negative; The cell proliferation index ki-67 positive cells were 3–5%.Conclusion Primary invasive fibromatosis of the stomach is a relatively rare spindle cell tumor, which needs to be differentiated from tumors and pathological lesions such as inflammatory myofibroblastic tumor, plexiform mucinous fibroma, gastrointestinal stromal tumor, etc.

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