Low-grade Malignancy Research Articles

Sertoliform endometrioid carcinoma of the right ovary.

Endometrioid carcinomas of the ovary are a subtype of epithelial ovarian tumors, with sertoliform endometrioid carcinomas being a rare variant. We report a case of a previously healthy premenopausal woman presenting with androgenic symptoms in the form of hirsutism and male pattern alopecia. On further testing, she was found to have high levels of luteinizing hormone and total testosterone levels, and imaging revealed a large pelvic abdominal mass in the right ovary. She underwent total hysterectomy with bilateral salpingo-oophorectomy. Microscopy and histopathology confirmed the diagnosis of sertoliform endometrioid carcinoma. Her symptoms improved significantly on follow-up. Androgenic tumors might not be common in premenopausal women; however, it is important to maintain a high level of suspicion in patients presenting with virilizing symptoms especially of rapid progression. Our 47-year-old patient presented with virilizing symptoms that were rapidly progressing, which raises the suspicion of an underlying androgen secreting neoplasm. Sertoliform endometrioid carcinoma (SEC) is an extremely rare variant of endometrioid carcinomas and tend to present at an earlier stage as compared to most endometrioid carcinomas of the ovary. Recognition of SEC in virilizing patients is important as it is a well-differentiated, low-grade malignancy with a good prognosis when confined to the ovary.

Neutrophil-, Monocyte- and Platelet-to-Lymphocyte Ratios, and Absolute Lymphocyte Count for Diagnosis of Malignant Soft-tissue Tumors.

Soft-tissue tumors are difficult to differentiate as benign or malignant. Immune markers, such as the neutrophil-lymphocyte ratio (NLR), monocyte-lymphocyte ratio (MLR), platelet-lymphocyte ratio (PLR), and absolute lymphocyte count (ALC) in serum, have been reported to be useful in the diagnosis and predicting prognosis of several malignancies. We investigated the diagnostic value of these immune markers in differentiating soft-tissue tumors. A total of 692 patients who underwent biopsy or surgery of soft-tissue tumors were included and divided into benign tumor, low-grade malignancy, or high-grade malignancy groups. Immune markers were calculated from the preoperative blood tests and compared between the groups. A receiver operating curve (ROC) analysis was conducted between the benign disease group and a combination of the groups with malignancy to determine which immune marker had the most diagnostic value. NLR and MLR were significantly different between the three groups with benign disease having the lowest value and high-grade malignancies the highest. Benign disease was also associated with lower PLR and higher ALC. There was no difference between the low- and high-grade malignancies in PLR and ALC. From the ROC analysis, NLR had the highest area under the curve (AUC) value of 0.773 out of the four markers. When limited to small tumors (≤30 mm), NLR had the highest AUC value of 0.729. The NLR showed the highest diagnostic value, although the diagnostic ability was not adequately high to differentiate benign and malignant soft-tissue tumors alone. NLR may serve as diagnostic support in combination with clinical history, physical findings, and tumor-imaging results.

A Decade of Therapeutic Challenges in Synchronous Gynecological Cancers from the Bucharest Oncological Institute.

The aim of our study is to present the particularities of a specific subset of gynecological cancer patients in Romania. We present a review of synchronous gynecological neoplasia (SGN) treated in the Bucharest Oncological Institute's surgery departments over a decade. Between 2012 and 2022, 7419 female patients with genital malignancies were treated. We identified 36 patients with invasive synchronous primary gynecological cancers (0.5%) and 12 cases with one primary gynecological and another primary invasive pelvic cancer (rectal/bladder). All recurrent, metastatic, or metachronous tumors detected were excluded. Demographic data, personal history, presenting symptoms, pathologic findings, staging, treatment, and evolution for each case were recorded. Usually, the most common SGN association is between ovarian and endometrial cancer of endometrioid differentiation (low-grade malignancies with very good prognosis). However, we noticed that, given the particularities of the Romanian medical system, the most frequent association is between cervical and endometrial, followed by cervical and ovarian cancers. Moreover, the cancer stage at diagnosis is more advanced. In countries with low HPV vaccination rate and low adherence to screening programs, SGNs can present as extremely advanced cases and require extensive surgery (such as pelvic exenterations) to achieve radicality. This multimodal treatment in advanced cases with high tumor burden determines a reduction in survival, time until progression, and quality of life.

A surgical conundrum: Managing primary tracheal adenoid cystic carcinoma with preservation of larynx

Title: A surgical conundrum: managing primary tracheal adenoid cystic carcinoma with preservation of larynx. Introduction: Tracheal tumors are extremely uncommon. The incidence of tracheal tumors is 0.09–0.2% out of which 10% are Adenoid cystic carcinoma. The origin of adenoid cystic carcinoma is from the submucosal glands of the tracheal tree. It is typically regarded as a low-grade malignancy with a favorable prognosis. Case Presentation: A 35 years old male patient came to Out Patient Department with chief complaints of change in voice since 6 months and stridor since 1 week. On Indirect laryngoscopy there was left sided vocal cord palsy with submucosal swelling in subglottic area compromising the airway. Contrast Enhanced Computed Tomography revealed a hyperintense lesion of about 5.5 cm craniocaudally in the posterolateral aspect of the subglottic trachea and inferior border of the cricoid pushing left thyroid lobe anterolaterally along with significant obstruction of the airway at the level of cricoid. An emergency tracheostomy was done at lower level to secure the airway. Flexible bronchoscopy showed a 1.5-2cm stenotic segment obstructing 50 to 75% of the airway in a subglottic region with left vocal cord palsy. A biopsy of the lesion took externally which came as an Adenoid Cystic Carcinoma. On Magnetic Resonance Imaging there was no involvement of the nerve or metastasis. Wide local excision of the lesion involving tracheal resection with cricotracheal anastomosis and Montgomery Tube insertion was done followed by chemoradiation. Montgomery tube was successfully removed and the patient satisfactorily decannulated. Conclusion: Adenoid cystic carcinoma should be considered as a differential diagnosis in patients with changes in voice and stridor. As adenoid cystic carcinoma is very slow-growing tumor so instead of being so radical a combined approach that gives a functional larynx and better quality of life should opt.

Extra-Parotid Pleomorphic Adenoma and Low-Grade Salivary Malignancy in the Head and Neck Region.

Background and objective Pleomorphic adenoma is the most common major salivary gland neoplasm. Around 80% of them arise in the parotid glands, whereas 10% originate in the minor salivary glands. The pleomorphic adenoma of the extra-parotid site is defined by its location outside the primary salivary gland. The minor salivary gland adenomas occur at the hard and soft palate, lips, tongue, lacrimal glands, pharynx, larynx, paranasal sinus, and nasal cavity. Pleomorphic adenoma in parapharyngeal space may occur de novo or as an extension of the deep lobe of the parotid tumors. Our objective in this study wasto assess the location and presentations of extra-parotid pleomorphic adenomaand frequency of low-grade salivary gland malignancy diagnosed as pleomorphic adenomavia fine-needle aspiration cytology (FNAC)in the head and neck region andthe treatment outcomes after the resection of the tumors. Materials and methods This was a retrospective observational study. All patients with FNAC-diagnosedpleomorphic adenoma of extra-parotid locations of the head and neck region who underwent curative surgery in the Department of Otorhinolaryngology and Head and Neck Surgery at a rural tertiary care center between August 1997 and August 2022 were included in the study. Data on the symptoms, FNAC report, surgical techniques, pathological results, adjuvant therapy, andany recurrence were documented. Data were entered into a Microsoft Excelsheet and analyzed using IBM SPSS Statistics version22 software (IBM Corp., Armonk, NY). Results Our study included 23 patients, of which 14 were females and nine were males. The various sites of involvementwere as follows: parapharyngeal space (four), larynx (one), nasal septum (two), hard palate (five), soft palate (four), hard and soft palate (three), and submandibular salivary gland (four). Of note, 17.3% of the patients had local recurrence withan average time frame of three years post-surgery: 20% in patients with low-grade malignancy and 16.6% in patients with pleomorphic adenoma. Conclusion Extra-parotid pleomorphic adenomas are common and have a high malignant potential. The preferred choice of treatment for extra-parotid salivary tumors is complete resection with adequate clearance. Malignant pleomorphic adenomas may require stagingneck dissection and adjuvant treatment for a better prognosis.

Kallikrein-Related Peptidase 12 (KLK12) in Breast Cancer as a Favorable Prognostic Marker.

Kallikrein-related peptides (KLKs) form an evolutionally conserved subgroup of secreted serine proteases that consists of 15 members (KLK1-15). Previous studies have shown that KLKs regulate diverse biological processes, but the clinical significance of KLKs remains largely unclear in human breast cancers. We examined the expression profile of 15 KLK genes in breast carcinomas using microarray data. Next, we immunolocalized KLK12 in 140 breast carcinomas and evaluated its clinical significance. Subsequently, we examined the effects of KLK12 on proliferation and migration in breast cancer cell lines. From microarray analyses, it turned out that KLK12 was the most strongly associated with low-grade malignancy in breast carcinomas among the 15 KLK members. Immunohistochemical KLK12 status was positively associated with ER and PR status, while it was inversely associated with stage, pathological T factor, lymph node metastasis, and distant metastasis. Prognostic analyses demonstrated that KLK12 was a favorable prognostic factor for both disease-free and breast cancer-specific survival of the patients. Furthermore, the knockdown of KLK12 significantly increased cell proliferation activity and cell migration of breast cancer cells. These results suggest that KLK12 has antitumorigenic effects associated with proliferation and migration and immunohistochemical KLK12 status as a potent favorable prognostic factor in breast carcinoma patients.

Characteristic imaging findings in a patient with chronic expanding hematoma on the floor of the mouth.

Chronic expanding hematoma (CEH) is defined as chronic hematoma enlargement for more than 1month. Although CEH rarely occurs on the floor of the mouth, the need to distinguish these cases from malignant disease is critical, given the need for potentially extensive resection in patients with malignancy. We report a case of CEH on the floor of the mouth, which required differentiation from malignant tumor. A 42-year-old woman was referred to our hospital for a submucosal mass on the right floor of the mouth, with a diagnosis of class 3 on aspiration cytology. Computed tomography revealed a submucosal mass with peripheral calcification on the floor of the mouth, which exhibited a hypointense rim on T2-weighted imaging and gradual nodular-like enhancement in the periphery on contrast-enhanced magnetic resonance imaging. Enucleation was performed to reach a definitive diagnosis, and CEH was confirmed pathologically. Well-defined morphology, presence of calcification, a hypointense rim on T2-weighted imaging, and weak peripheral nodular-like enhancement may be characteristic findings of CEH on the floor of the mouth. Accordingly, these imaging features may aid in differentiating CEH from low-grade malignancies and in determining the optimal management strategy.

Salivary Gland Secretory Carcinoma: Clinicopathologic and Genetic Characteristics of 215 Cases and Proposal for a Grading System.

Salivary gland secretory carcinoma (SC), previously mammary analog SC, is a low-grade malignancy characterized by well-defined morphology and an immunohistochemical and genetic profile identical to SC of the breast. Translocation t(12;15)(p13;q25) resulting in the ETV6::NTRK3 gene fusion is a characteristic feature of SC along with S100 protein and mammaglobin immunopositivity. The spectrum of genetic alterations for SC continues to evolve. The aim of this retrospective study was to collect data of salivary gland SCs and to correlate their histologic, immunohistochemical, and molecular genetic data with clinical behavior and long-term follow-up. In this large retrospective study, we aimed to establish a histologic grading scheme and scoring system. A total of 215 cases of salivary gland SCs diagnosed between 1994 and 2021 were obtained from the tumor registries of the authors. Eighty cases were originally diagnosed as something other than SC, most frequently acinic cell carcinoma. Lymph node metastases were identified in 17.1% (20/117 cases with available data), with distant metastasis in 5.1% (6/117). Disease recurrence was seen in 15% (n=17/113 cases with available data). The molecular genetic profile showed ETV6::NTRK3 gene fusion in 95.4%, including 1 case with a dual fusion of ETV6::NTRK3 and MYB::SMR3B. Less frequent fusion transcripts included ETV6::RET (n=12) and VIM::RET (n=1). A 3-tiered grading scheme using 6 pathologic parameters (prevailing architecture, pleomorphism, tumor necrosis, perineural invasion (PNI), lymphovascular invasion (LVI), and mitotic count and/or Ki-67 labeling index) was applied. Grade 1 histology was observed in 44.7% (n=96), grade 2 in 41.9% (n=90), and grade 3 in 13.5% (n=29) of cases. Compared with low-grade and intermediate-grade SC, high-grade tumors were associated with a solid architecture, more prominent hyalinization, infiltrative tumor borders, nuclear pleomorphism, presence of PNI and/or LVI, and Ki-67 proliferative index >30%. High-grade transformation, a subset of grade 2 or 3 tumors, seen in 8.8% (n=19), was defined as an abrupt transformation of conventional SC into high-grade morphology, sheet-like growth, and a tumor lacking distinctive features of SC. Both overall survival and disease-free survival (5 and 10y) were negatively affected by tumor grade, stage, and TNM status (each P<0.0001). SC is a low-grade malignancy with predominantly solid-microcystic growth patterns, driven by a gene fusion, most commonly ETV6::NTRK3. There is a low risk for local recurrence and a good overall long-term survival, with a low risk for distant metastasis but a higher risk for locoregional lymph node metastasis. The presence of tumor necrosis, hyalinization, PNI and/or LVI, and positive resection margins correlate with higher tumor grade, less favorable prognosis, and increased mortality. The statistical results allowed us to design a 3-tiered grading system for salivary SC.

The first case reported in Southeast Asia of Huge Frantz’s Tumor

Introduction: Solid pseudopapillary pancreatic tumor (Frantz’s Tumor) is a low-grade malignancy, that lacks clinical symptomatology. It was treatable with a favorable prognosis. Case presentation: A young female patient presented with increasing abdominal pain three years ago. Abdominal examination revealed a lump palpable. Contrast-enhanced Computed Tomography abdomen reported a solid mass measuring 13.9 cm x 13.2 cm x 8.9 cm from the pancreas with an enhancing cystic component. Fine-needle aspiration biopsy yielded a cellular sample comprising pseudopapillary neoplasm of the pancreas. A distal pancreatectomy was performed; she was discharged in satisfactory condition.

Abstract 3302: KLK7 expression in human tumors: A tissue microarray study on 13,447 tumors

Abstract Background: Kallikrein-related peptidase 7 (KLK7) is a chymotrypsin-like serine protease which is essential for the desquamation of corneocytes and thus plays a pivotal role in maintaining skin homeostasis. In cancer, KLK7 overexpression was suggested to represent a route for metastasis through cleavage of cell junction and extracellular matrix proteins of cancer cells. Methods: To comprehensively determine KLK7 protein expression in normal and neoplastic tissues, a tissue microarray containing 13,447 samples from 147 different tumor types and subtypes as well as 608 samples of 76 different normal tissue types was analyzed by immunohistochemistry. Results: KLK7 positivity was found in 64 of 147 tumor categories, including 17 tumor categories with at least one strongly positive case. The highest rate of KLK7 positivity was found in squamous cell carcinomas from various sites of origin (positive in 18.1%-63.8%), ovarian and endometrium cancers (4.8%-56.2%), salivary gland tumors (4.8%-13.7%), bilio-pancreatic adenocarcinomas (20.0%-40.4%), and adenocarcinomas of the upper gastrointestinal tract (3.3%-12.5%). KLK7 positivity was linked to nodal metastasis (p=0.0005), blood vessel infiltration (p=0.0037), and lymph vessel infiltration (p&amp;lt;0.0001) in colorectal adenocarcinoma, nodal metastasis in hepatocellular carcinoma (p=0.0382), advanced pathological tumor stage in papillary thyroid cancer (p=0.0132), and low grade of malignancy in a cohort of 719 squamous cell carcinomas from 11 different sites of origin (p&amp;lt;0.0001). Conclusions: These data provide a comprehensive overview on KLK7 expression in normal and neoplastic human tissues. The prognostic relevance of KLK7 expression and the possible role of KLK7 as a drug target need to be further investigated. Citation Format: Simon Kind, Carolina Castillo, Ria Uhlig, Natalia Gorbokon, Maximilian Lennartz, Sebastian Dwertmann Rico, Viktor Reiswich, Florian Viehweger, Martina Kluth, Claudia Hube-Magg, Christian Bernreuther, Franziska Büscheck, Till Clauditz, Christroph Fraune, Andrea Hinsch, Till Krech, Patrick Lebok, Stefan Steurer, Eike Burandt, Sarah Minner, Andreas Marx, Ronald Simon, Waldemar Wilczak, Guido Sauter, Anne Menz, Frank Jacobsen. KLK7 expression in human tumors: A tissue microarray study on 13,447 tumors [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2023; Part 1 (Regular and Invited Abstracts); 2023 Apr 14-19; Orlando, FL. Philadelphia (PA): AACR; Cancer Res 2023;83(7_Suppl):Abstract nr 3302.

Sclerosing Central Mucoepidermoid Carcinoma: Rare Case Series and Review

Introduction:&#x0D; The most frequent malignant tumour of the salivary glands is mucoepidermoid carcinoma which accounts for 30% of all malignancies of the salivary glands. There are numerous histologic variations with various levels of differentiation. Sclerosing MEC (SMEC) is a rare subtype of MEC that has the potential to be misdiagnosed as a low-grade non-SMEC malignancy or a benign reactive condition. There have been no cases of the extremely rare sclerosing variant of central or intraosseous MEC reported as of yet.&#x0D; Case reports:&#x0D; We present two cases of Sclerosing Central MEC, in which histologic analysis revealed relatively well-circumscribed, nonencapsulated tumours made up of dispersed epithelial islands of low-grade MEC and extensive central sclerosis. Similar sclerotic stroma was present in the tumor in the second case, but the epithelial component was of intermediate grade. Both cases showed abundant intracytoplasmic mucin and positive Mayer mucicarmine and PAS stains.&#x0D; Conclusions:&#x0D; Sclerosing central mucoepidermoid carcinoma was identified as the cause. Both cases underwent a complete resection of the tumor and have since been free of the disease.

A description of Kaposi sarcoma risk factors and outcomes in HIV-positive and HIV-negative patients at a tertiary care medical center from 2005 to 2020.

Kaposi sarcoma (KS) is a low-grade vascular malignancy caused by human herpesvirus-8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus (KSHV). There are four established subtypes of KS, which are described by unique risk factors, presentation, and disease course. A "non-epidemic" variant to describe HIV-negative men who have sex with men (MSM) is emerging as a fifth subtype. We retrospectively examined patients with KS at an academic medical center in central Ohio, USA. To our knowledge, this is the first US-based report to describe KS risk factors and outcomes in the context of HIV status. Data were extracted from patient charts including demographic information, history at time of KS diagnosis, and information about KS disease course. HIV-positive and HIV-negative patients were grouped into established categories. HIV-negative patients who did not fit an existing subtype were described as "Unclassified-KS-Type." Demographic characteristics for AIDS-KS patients in our cohort match established trends in this subtype, such as male, MSM, and younger age at diagnosis compared to HIV-negative patients. Most Unclassified-KS-Type patients fit well into the emerging "non-epidemic KS" subtype. These patients are described as healthy, middle-aged, HIV-negative MSM with lower extremity lesions. This descriptive report provides an updated view of KS risk factors and outcomes to improve detection and treatment in dermatology.

Inflammatory myofibroblastic tumour of the common bile duct: a case report and literature review

IntroductionInflammatory myofibroblastic tumour (IMT) of the common bile duct (CBD) is an extremely rare low-grade malignancy with various biological behaviours and a lack of specific clinical and histopathological features. Preoperative and intraoperative diagnosis are challenging, and a diagnostic delay may increase surgical complexity.Case presentationWe present the case of a 34-year-old male with no relevant medical history who presented with jaundice of 20 days of evolution. Histology and immunohistochemistry confirmed the diagnosis of an IMT with anaplastic lymphoma kinase (ALK)-1 expression. In addition, a review of the relevant literature revealed 13 published reports of biliary IMTs. The clinical history and histopathological features in these 13 cases were compared with those in our case to provide a comprehensive overview of the clinical manifestations and histopathological features of the disease.ConclusionIMT of the CBD is an extremely rare low-grade malignancy that mainly occurs in middle-aged female patients. The main clinical manifestation is monosymptomatic jaundice. The reported tumours originated in the middle and lower segments of the CBD, with an average size of approximately 3.5 cm × 3.0 cm and tumour cells expressing smooth muscle actin (SMA), vimentin and ALK. Abnormal ALK expression and ALK gene rearrangement represent potential histopathological and differential diagnoses. A clear diagnosis by preoperative biopsy and intraoperative frozen section examination is critical and can significantly reduce surgical trauma. The prognosis is good, and very few patients experience recurrence or distant metastasis.

Solid Pseudopapillary Neoplasms of the Pancreas: Clinicopathologic Analysis and a Predictive Model

Solid pseudopapillary neoplasms (SPNs) of the pancreas are rare. They are considered low-grade malignancies, and a small percentage of patients experience recurrence or metastasis. It is critical to investigate associated biological behavior and identify patients at a risk of relapse. This was a retrospective study of 486 patients with SPNs who were diagnosed between 2000 and 2021. Their clinicopathologic features, including 23 parameters and prognoses were analyzed. Six patients (1.2%) presented with synchronous liver metastasis. A total of 21 patients experienced recurrence or metastasis postoperatively. The overall and disease-specific survival rates were 99.8% and 100%, respectively. The 5- and 10-year relapse-free survival (RFS) rates were 97.4% and 90.2%, respectively. Tumor size, lymphovascular invasion, and the Ki-67 index were independent predictors of relapse. Furthermore, a Peking Union Medical College Hospital–SPN risk model was built to evaluate the risk of relapse and compared it with the American Joint Committee on Cancer tumor staging system (eighth edition, 2017). Risk factors included 3 parameters: tumor size (>9 cm), lymphovascular invasion status (presence), and Ki-67 index (>1%). Risk grades were available for 345 patients, who were divided into 2 groups: (1) low risk (n = 124) and (2) high risk (n = 221). The group with no risk factors was designated as low risk and had a 10-year RFS of 100%. The group associated with 1 to 3 factors was designated as high risk, with a 10-year RFS of 75.3%. Receiver operating characteristic curves were generated, and the area under the curve was 0.791 for our model and 0.630 for the American Joint Committee on Cancer with respect to the cancer staging system. We validated our model in independent cohorts and demonstrated a sensitivity of 98.3%. In conclusion, SPNs are low-grade malignant neoplasms that rarely metastasize, and the 3 selected pathologic parameters can be used to predict their behavior. A novel Peking Union Medical College Hospital–SPN risk model was proposed for routine application to guide the patient counseling in clinical practice.

A Single Institution Experience with Solid Pseudopapillary Neoplasm of the Pancreas: Clinicopathological Correlation and Review of the Literature

Introduction: SPN is a rare, low-grade malignancy that accounts for 1-3% of all pancreatic neoplasms and tends to affect young females. It can be asymptomatic or present with abdominal pain, and surgical resection is the only curative treatment. Methods: A retrospective study was conducted on patients with biopsy proven Solid Pseudopapillary Neoplasm (SPN) of the pancreas between January 2016 and June 2021. The study included 14 cases, including 7 resection specimens and 7 biopsies, and was conducted at a tertiary referral center in PakistanResults: All 14 patients were female and ranged in age from 15 to 48 years old, with a mean age of 24. The most common symptom was abdominal pain, and the head of the pancreas was the most common location of the tumor. Tumor size ranged from 5 cm to 18 cm, and one patient had lymph node involvement and two patients had hepatic metastasis. Most patients received surgical treatment, and follow-up information was available for 8 out of the 14 patients, who were all alive with no recurrence or metastasis. Conclusion: Due to its rarity, non-specific clinical presentation, and imaging findings, it can be challenging to diagnose. There is a lack of data on SPN from South Asia, so this study provides valuable insights into the occurrence and treatment of SPN in this region.

A Case of Endocrine Mucin-Producing Sweat Gland Carcinoma of the Eyelid

Purpose: We report an endocrine mucin-producing sweat gland carcinoma diagnosed by biopsy in a patient who presented with an eyelid mass.Case summary: A 64-year-old male presented with a 3 × 3 mm solitary, painless pinkish mass on the right lower eyelid that had developed over the past year. The mass was excised and a biopsy was performed. The pathological findings included basaloid nodules composed of cells with eosinophilic cytoplasm, focal mucin production, and occasional glandular structures. Immunohistochemical examination was positive for cytokeratin 7 (CK-7), tumor protein 63 (P63), and the androgen receptor (AR). The patient was thus diagnosed with an endocrine mucin-producing sweat gland carcinoma. The lesion healed, and there has been no sign of recurrence over 6 months of follow-up.Conclusions: An endocrine mucin-producing sweat gland carcinoma is a very rare low-grade glandular malignancy that has not been reported in Korea previously. The prognosis is good (i.e., there is no recurrence) when the lesion is completely surgically excised. We thought it would be useful to report this very rare case.

Epithelioid haemangioendothelioma: case report and brief revision of a rare vascular tumour

We describe the case of a 71-year-old man who presented with a single painful soft tissue mass of the right thigh. Following imaging and subsequent excision, histological and immunohistochemical assessment of the tumour was performed, and the diagnosis of epithelioid haemangioendothelioma (EHE) was made. EHE is a rare translocation-related vascular sarcoma with variable clinical behaviour ranging from a low grade malignancy with ability to recur locally, to that of a high grade sarcoma, with propensity for systemic involvement.

Survival benefit stratification of partial nephrectomy versus non-surgical treatment in elderly patients with T1a renal cell carcinoma.

Renal cell carcinoma (RCC) of stage T1a has been proven to be of low-grade malignancy and mostly affects elderly individuals with relatively limited life expectancy. However, research on the survival benefit of surgery relative to non-surgical treatment (NST) is limited. The aim of the study was to investigate the survival difference between partial nephrectomy (PN) and NST and to establish a benefit stratification model for elderly patients (≥70 years) diagnosed with T1a RCC. Patients diagnosed with non-metastatic T1a RCC who received PN or NST were identified from the SEER database during 2004-2015. Before survival analysis, propensity score matching (PSM) was performed. Overall survival (OS) was estimated by the Kaplan-Meier method, and subgroup analyses were used to identify favorable factors of PN. Independent factors of survival were recognized by multivariate Cox regression analysis. Patients diagnosed with non-metastatic T1a RCC who received PN or NST were identified from the SEER database during 2004-2015. Before survival analysis, propensity score matching (PSM) was performed. Overall survival (OS) was estimated by the Kaplan-Meier method, and subgroup analyses were used to identify favorable factors of PN. Independent factors of survival were recognized by multivariate Cox regression analysis. Our findings suggest that the survival benefit of PN could be stratified based on the clinical characteristics in patients with stage T1a RCC aged 70 years or older, which may help physicians and patients optimize clinical decisions.

Milan System for Reporting Salivary Gland Cytopathology- An overview with Interobserver Variability and Clinicoradiological Concordance

Introduction: Salivary gland lesions are one of the most heterogenous lesions with overlapping features between low grade malignancy and benign lesions thus making them one of the most difficult areas for diagnosing. Cytology of salivary gland lesions plays an extremely important part in diagnosing the lesions and categorising them into non neoplastic, benign and malignant neoplasms, thereby allowing the patients and clinicians to make an accurate decision regarding whether the lesion is followed, biopsied, excised or a radical operation might be needed. Aim: To classify salivary gland lesions according to MILAN System for Reporting Salivary Gland Cytopathology (MSRSGC). Materials and Methods: This observational study was conducted from June 2021 to May 2022 where Fine Neddle Aspiration Cytology (FNAC) of salivary gland lumps was done on patients presenting to the Department of Pathology Rohilkhand Medical College and Hospital, Bareilly. The clinical and radiological features were noted. The cases were diagnosed on conventional cytopathology. Additionally categorisation as per MILAN System was done by two pathologists independently. The results were compared with histopathology, where available. Data was collected, entered and compiled in Microsoft excel followed by analysis using the software Statistical Package for Social Sciences (SPSS) version 23.0. The data was represented in frequency and further analysed using Kappa statistics. Also the validity was calculated in terms of sensitivity, specificity, Positive Predictive Value and Negative Predictive Value (NPV). Results: Total of 60 cases were included with M:F ratio as 1.6:1. Most common age group was third decade with 17 (28.3%) cases, closely followed by fourth decade with 11 (18.3%) cases. Most common salivary gland to be affected was parotid gland with 30 (50%) cases. Most common MILAN category was II, non neoplastic with 26 (43.3%) cases, followed by IV A, benign neoplasm with 19 (31.67%) cases. The Cohen kappa coefficient was 0.952 which showed a near perfect agreement between the two pathologists. The sensitivity, specificity, PPV and NPV and accuracy was 75%, 92.8%, 75% and 92.8%, 89% respectively. The Risk of Malignancy (ROM) for category I,II,III,IVA,VI was 0%, 0%, 50%, 9%, 100% respectively. Conclusion: The MILAN System for Reporting Salivary Gland Cytopathology (MSRSGC) offers a structured reporting system. The terminologies are reproducible and convey clear meaning among all the medical professionals including different pathologists and treating physician or surgeon and guide in deciding the accurate treatment based on the ROM for different categories.

Retroperitoneal Cystic Lymphangioma : case report

Lymphangiomas, cystic hygroma or macrocystic lymphatic malformation are a rare and benign congential malformations of the lymphatic system. Adults are affected rarely and less than children, and the abdominal location even less. Here, we discuss the case of a 56years old woman admitted and operated for a 7cm cystic mass of the tail of the pancreas, whom per operative exploration showed a retroperitoneal polycystic mass and histopathology concluded a lymphangioma. Less than 1% affect the retroperitoneum, they typically occur in spaces surrounded by loose connective tissue, the are difficult to differentiate from a mucinous cystic neoplasm of the pancreas by imaging since both have the appearance of multilocular cysts. Ultrasonography is very sensitive and relatively specific for evaluation of abdominal cystic masses. Laparoscopic removal has certain advantages over classical laparotomy, complete excision is recommended to protect the patient from subsequent recurrences even though cystic lymphangiomas are low-grade malignancies. The diagnosis of CL often is facilitated by means of modern imaging. Differential diagnosis include pancreatic pseudocysts, mucinous and serous cystadenomas, other congenital cysts and pancreatic ductal carcinoma with cystic degeneration. If symptomatic lesions or complications arise, complete surgical excision is a must.