BACKGROUND

Primary cancers of the appendix are rare, with an incidence of approximately 1.2 cases per 100,000 people per year and this tumor is difficult to diagnose preoperatively. The purpose of this paper is to present a rare case of metastatic mucinous carcinoma of the appendix and to provide a high index of suspicion to patients presenting with the same history, signs, and symptoms.

We present a case of a 33-year-old Filipina who reported abdominal pain and right lower quadrant mass. Following several preoperative diagnostic tests, a colonoscopy revealed synchronous tumors in various locations, prompting the need for an exploratory laparotomy to evaluate the abdomen. Histopathological examination was performed to confirm the final diagnosis which revealed primary mucinous carcinoma of the appendix. The tumor had extended into adjacent structures, including the cecal colon, ileum, and right ureter. Metastatic lesions were also identified in the descending and sigmoid colon. The disease was classified as stage IVC (T4b, N1c, M1c), indicating advanced progression with both extensive local invasion and distant metastasis.

Histopathology remains the gold standard for cancer diagnosis. Given the rarity and complexity of appendiceal mucinous carcinoma, a multidisciplinary approach is also essential. This collaborative strategy from various specialties is vital not only for achieving an accurate diagnosis but also for developing and implementing an effective, individualized treatment plan that addresses the distinct challenges of this uncommon malignancy.

Krukenberg tumors are very rare. Its origin is difficult to define especially if its gross features mimic a primary ovarian cancer. We present a case of a 24-year-old Filipino female patient with metastatic mucinous ovarian adenocarcinoma of colonic origin that mimicked primary ovarian cancer and genitourinary tuberculosis. Surgery was done and histopathology revealed that the cancer was a metastatic mucinous adenocarcinoma of colonic origin. This case highlights the importance of differentiating between benign and malignant ovarian lesions as well as distinction between primary and metastatic ovarian neoplasms. Radiological imaging has an evolving role in diagnosis of different cancers, which may be improved through better clinical correlation and developing meaningful differential diagnosis while advancing to a more strategized algorithm in the diagnostic approach.

Objective: To explore the feasibility and application value of intraoperative direct immunohistochemical (IHC) staining in improving the diagnosis accuracy in difficult cases of bronchiolar adenoma (BA). Methods: Nineteen cases with single or multiple pulmonary ground-glass nodules or solid nodules indicated by imaging in Cancer Hospital of Chinese Academy of Medical Sciences from January to July 2021 and with difficulty in differential diagnosis at frozen HE sections were selected. In the experimental group, direct IHC staining of cytokeratin 5/6 (CK5/6) and p63 was performed on frozen sections to assist the differentiation of BA from in situ/micro-invasive adenocarcinoma/adenocarcinoma/invasive mucinous adenocarcinoma. In the control group, two pathologists performed routine frozen HE section diagnosis on these 19 cases. The diagnostic results of paraffin sections were used as the gold standard. The sensitivity and specificity of BA diagnosis, consistency with paraffin diagnosis and time used for frozen diagnosis were compared between the experimental group and the control group. Results: The basal cells of BA were highlighted by CK5/6 and p63 staining. There were no basal cells in the in situ/microinvasive adenocarcinoma/adenocarcinoma/invasive mucinous adenocarcinoma. In the experimental group, the sensitivity and specificity with aid of direct IHC staining for BA were 100% and 86.7%, respectively, and the Kappa value of frozen and paraffin diagnosis was 0.732, and these were significantly higher than those in the control group (P<0.05). The average time consumption in the experimental group (32.4 min) was only 7 min longer than that in the control group (25.4 min). Conclusions: Direct IHC staining can improve the accuracy of BA diagnosis intraoperatively and reduce the risk of misdiagnosis, but require significantly longer time. Thus frozen direct IHC staining should be restricted to cases with difficulty in differentiating benign from malignant diseases, especially when the surgical modalities differ based on the frozen diagnosis.
Humans ; Paraffin ; Sensitivity and Specificity ; Adenocarcinoma in Situ ; Adenoma/diagnosis* ; Adenocarcinoma, Mucinous/surgery* ; Frozen Sections/methods*

Humans ; Adenocarcinoma, Mucinous/pathology* ; Stomach Neoplasms/pathology*

Objective: To summarize the clinical characteristics of patients with skip metastasis at esophageal resection margin during radical gastrectomy. Methods: This is a descriptive study of case series. Relevant data from 2006 to 2022 were collected from two major gastric cancer consultation and treatment centers: Nanjing Drum Tower Hospital and Jinling Hospital.Characteristics, surgical approach, number of dissected lymph nodes, immunohistochemical staining, and pathological staging were summarized and analyzed. The distribution of residual tumor cells at the esophageal margins was further analyzed at the cellular and tissue levels. Skip metastasis at the esophageal resection margin was defined as a negative esophageal margin with a positive margin in the cephalad donut. Results: Thirty (0.33%, 30/8972) eligible patients, 24 (80.0%) of whom were male, were identified in the two centers. The mean age was 63.9±11.0 years. Seventeen (56.7%) of these patients had papillary or tubular adenocarcinomas, including 13 (43.3%) poorly- and four (13.3%) moderately-differentiated tumors; four (13.3%) had signet-ring cell carcinomas; four (13.3%) mucinous adenocarcinomas; three (10.0%) mixed adenocarcinomas, including two with poorly-differentiated tubular adenocarcinomas mixed with signet-ring cell carcinoma and mucinous adenocarcinoma; and one had a poorly differentiated tubular adenocarcinoma mixed with signet-ring cell carcinoma. Two patients (6.7%) had other types of cancer, namely adenosquamous carcinoma in one patient and undifferentiated carcinoma in the other one. The predominant tumor sites were the lesser curvature (n=26, 86.7%) and the cardia (n=24, 80.0%). The mean tumor diameter was 6.6 cm, mean distance between tumor and esophageal resection margin was 1.5 cm, and proportions of tumor invasion into the dentate line, nerves, and vessels were 80.0% (24/30), 86.7%(26/30), and 93.3% (28/30), respectively. The mean number of lymph nodes resected was 20.4±8.9. The pathological stage was mainly T4 (n=18, 60.0%) and N3 (n=21, 70.0%), the median Ki67 was 52.7%, and the rates of positivity for HER2, EGFR, VEGFR, E-cadherin and PD-L1 were 40.0% (12/30), 46.7% (14/30), 80.0% (24/30), 86.7% (26/30) and 16.7% (5/30), respectively. At the cellular level, cancer cells were mainly distributed in small focal areas, as cell masses, or as tumor thrombi; large numbers of widely distributed atypic cells were seldom observed. At the tissue level, cancer cells were located in the mucosal layer in seven patients (23.3%), in the submucosal layer in 18 (60.0%), and in the muscular layer in five (16.7%); no cancer cells were identified in the outer membrane. Five of the seven tumors were located in the lamina propria, two in the muscularis mucosae, and none in the mucosal epithelium. Conclusion: Patients with skip metastasis at the esophageal resection margin at radical gastrectomy have unfavorable tumor biology and a high proliferation index, are at a late pathological stage, and the residual cancer is mostly located in the submucosa.
Humans ; Male ; Middle Aged ; Aged ; Female ; Margins of Excision ; Adenocarcinoma/pathology* ; Carcinoma, Signet Ring Cell/pathology* ; Lymph Nodes/pathology* ; Adenocarcinoma, Mucinous/pathology* ; Stomach Neoplasms/pathology* ; Gastrectomy ; Neoplasm Staging ; Retrospective Studies

OBJECTIVE: To investigate and summarize the clinicopathological features, immunophenotype, differential diagnosis and prognosis analysis of mucinous tubular and spindle cell carcinoma (MTSCC). METHODS: The data of thirteen cases of MTSCC were retrospectively analyzed, the clinical and pathological characteristics and immunohistochemical expression were summarized, and fluorescence in situ hybridization was detected. RESULTS: Among the thirteen patients, four were males and nine females, with a male-to-female ratio of 1 ∶2.25. The average age was 57.1 years, ranging from 39 to 78 years. The maximum diameter of the tumor was 2-12 cm. All cases had no symptoms, and were accidentally discovered, 3 cases underwent partial renal resection, 10 cases underwent radical renal resection, 9 cases were located in the left kidney, and 4 cases were located in the right kidney. Most of the cases showed the classical morphological changes, with 11 cases of nuclear grading [World Health Organization (WHO)/International Society of Urological Pathology (ISUP) grading system] being G2 and 2 cases being G3. There were 6 cases of stage PT1a, 3 cases of PT1b, 2 cases of PT2a, and 1 case of PT2b and 1 case of PT3a. The positive rates of immunohistochemical staining were: vimentin, AE1/AE3, α-methylacyl-CoA racemase (αMACR) and cytokeratin (CK) 8/18, 100% (13/13); CK7, 92.3% (12/13); epithelial membrane antigen (EMA), 92.3% (12/13); CK20, 46.2% (6/13); CD10, 30.8% (4/13); synaptophysin (Syn), 7.7% (1/13); chromogranin A (CgA), CD57, WT1 and Ki-67, 0 (0/13), and fluorescence in situ hybridization showed that no trisomy of chromosomes 7 and 17 were observed in any of the cases. The follow-up period was 6 months to 7 years and 6 months, 2 cases died after lung metastasis (one with ISUP/WHO grade G3, one with necrosis), and the remaining 11 cases had no recurrence and metastasis. CONCLUSION MTSCC is a unique type of low-grade malignancy kidney tumor, occurs predominantly in females, widely distributed in age, the current treatment method is surgical resection, and cases with necrosis and high-grade morphology are prone to recurrence and metastasis, although most cases have a good prognosis, but they still need close follow-up after surgery.
Humans ; Male ; Female ; Middle Aged ; Kidney Neoplasms/surgery* ; Carcinoma, Renal Cell/diagnosis* ; In Situ Hybridization, Fluorescence ; Retrospective Studies ; Adenocarcinoma, Mucinous/pathology* ; Kidney/pathology* ; Prognosis ; Necrosis

Objective: To analyzed perioperative safety of cytoreductive surgery (CRS) for patients with colorectal cancer peritoneal metastasis (CRPM) and to construct a predictive model for serious advese events (SAE). Methods: A descriptive case-series study was conducted to retrospectively collect the clinicopathological data and treatment status (operation time, number of organ resection, number of peritoneal resection, and blood loss, etc.) of 100 patients with peritoneal metastases from colorectal cancer or appendix mucinous adenocarcinoma who underwent CRS at the Sixth Affiliated Hospital of Sun Yat-sen University from January 2019 to August 2021. There were 53 males and 47 females. The median age was 52.0 (39.0-61.8) years old. Fifty-two patients had synchronous peritoneal metastasis and 48 had metachronous peritoneal metastasis. Fifty-two patients received preoperative neoadjuvant therapy. Primary tumor was located in the left colon, the right colon and the rectum in 43, 28 and 14 cases, respectively. Fifteen patients had appendix mucinous adenocarcinoma. Measures of skewed distribution are expressed as M (range). Perioperative safety was analyzed, perioperative grade III or higher was defined as SAE. Risk factors associated with the occurrence of SAEs were analyzed using multivariate logistic regression. A nomogram was plotted by R software to predict SAE, the efficacy of which was evaluated using the area under the ROC curve (AUC) and correction curves. Results: The median peritoneal cancer index (PCI) score was 16 (1-39). Sixty-eight (68.0%) patients achieved complete tumor reduction (tumor reduction score: 0-1). Sixty-two patients were treated with intraperitoneal hyperthermic perfusion chemotherapy (HIPEC). Twenty-one (21.0%) patients developed 37 SAEs of grade III-IV, including 2 cases of ureteral injury, 6 cases of perioperative massive hemorrhage or anemia, 7 cases of digestive system, 15 cases of respiratory system, 4 cases of cardiovascular system, 1 case of skin incision dehiscence, and 2 cases of abdominal infection. No grade V SAE was found. Multivariate logistic regression analysis showed that CEA (OR: 8.980, 95%CI: 1.428-56.457, P=0.019), PCI score (OR: 7.924, 95%CI: 1.486-42.259, P=0.015), intraoperative albumin infusion (OR: 48.959, 95%CI: 2.115-1133.289, P=0.015) and total volume of infusion (OR: 24.729, 95%CI: 3.956-154.562, P=0.001) were independent risk factors for perioperative SAE in CRS (all P<0.05). Based on the result of multivariate regression models, a predictive nomogram was constructed. Internal verification showed that the AUC of the nomogram was 0.926 (95%CI: 0.872-0.980), indicating good prediction accuracy and consistency. Conclusions: CRS is a safe and effective method to treat CRPM. Strict screening of patients and perioperative fluid management are important guarantees for reducing the morbidity of SAE.
Adenocarcinoma, Mucinous/therapy* ; Adult ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Appendiceal Neoplasms/surgery* ; Colorectal Neoplasms/pathology* ; Combined Modality Therapy ; Cytoreduction Surgical Procedures/methods* ; Female ; Humans ; Hyperthermia, Induced/methods* ; Male ; Middle Aged ; Peritoneal Neoplasms/secondary* ; Retrospective Studies ; Survival Rate

Objective: To explore the application value of diffusion kurtosis imaging (DKI) in the differential diagnosis of rectal tumors and evaluating the prognostic factors associated with rectal adenocarcinoma. Methods: A total of 105 patients with rectal tumors admitted in the First Affiliated Hospital of Zhengzhou University from December 2018 to August 2020 were retrospectively analyzed. All patients underwent high-resolution magnetic resonance DKI scanning. The mean diffusivity (MD), mean kurtosis (MK) and apparent diffusion coefficient (ADC) were measured and the relationship of these parameters with pathological types and prognostic factors of rectal tumor were analyzed. The diagnostic efficacy of MD, MK, and ADC for positive circumferential resection margin (CRM) and extramural venous invasion (EMVI) of rectal adenocarcinoma was evaluated by the receiver operating characteristic (ROC) curve. Results: MD and ADC were only related to pathological type. The MD and ADC were (2.091±0.390)×10(-3) and (1.478±0.265)×10(-3) mm(2)/s in mucinous adenocarcinoma, higher than (1.136±0.182)×10(-3) and (0.767±0.077)×10(-3) mm(2)/s in unspecified adenocarcinoma and (1.617±0.697)×10(-3) and (0.940±0.179)×10(-3) mm(2)/s in tubulo-villous adenoma. The MD and ADC in unspecified adenocarcinoma were lower than those in tubule-villous adenoma (P<0.05). Nevertheless, MK was associated with pathological type, N stage, CRM and EMVI. The MK was 0.566±0.110 in mucinous adenocarcinoma, lower than 0.982±0.135 in unspecified adenocarcinoma and 0.827±0.121 in tubulo-villous adenoma. The MK in unspecified adenocarcinoma was higher than that in intubulo-villous adenoma. The MK was 0.984±0.107 in pN1-2, higher than 0.881±0.146 in pN0. The MK was 0.990±0.142 in positive CRM, higher than 0.862±0.114 in negative CRM. The MK was 0.996±0.140 in positive EMVI, higher than 0.832±0.100 in negative EMVI (P<0.05). The ROC curves showed that the AUCs of MD, MK and ADC in diagnosing positive CRM were 0.459, 0.653 and 0.408, respectively; with MK=1.006 as the optimal diagnostic threshold, the diagnostic sensitivity and specificity were 51.9% and 81.0%, respectively. The AUCs of MD, MK and ADC values in diagnosing positive EMVI were 0.510, 0.662 and 0.388, respectively; with MK=1.010 as the optimal diagnostic threshold, the diagnostic sensitivity and specificity were 50.9% and 87.5%, respectively. Conclusions: DKI quantitative parameter is helpful for discriminating rectal tubulo-villous adenoma, unspecified adenocarcinoma, and mucinous adenocarcinoma, and is helpful for predicting the prognosis of patients with rectal adenocarcinoma. High MK is associated with positive CRM and EMVI.
Humans ; Adenocarcinoma/diagnostic imaging* ; Adenocarcinoma, Mucinous/diagnostic imaging* ; Adenoma, Villous/diagnostic imaging* ; Diffusion Magnetic Resonance Imaging/methods* ; Prognosis ; Rectal Neoplasms/pathology* ; Retrospective Studies ; Sensitivity and Specificity

Objective: To investigate the clinicopathological features, surgical methods and prognosis of primary appendiceal neoplasms. Methods: A descriptive case series study was performed. Clinical data of patients diagnosed with primary appendiceal neoplasms in Peking University First Hospital from 2006 to 2017 were retrospectively analyzed. Patients who underwent surgery and were confirmed as appendiceal neoplasms by postoperative pathology were included. Cases of cecal tumor invading the appendix and other organ tumors implanting in the appendix and cases of recurrent appendix tumors were excluded Pathological classification was based on the 4th edition of the WHO classification of digestive tract tumors (2010 edition), and the efficacy of operation methods of low grade appendiceal mucinous neoplasm (LAMN) were analyzed. Results: A total of 115 patients were enrolled, including 52 males and 63 females with a median age of 59 (51, 71) years. Clinical symptoms usually manifested as dormant pain in the right lower quadrant, migrating right lower abdominal pain, fever and bloating. Twenty-four cases were accidentally discovered during surgery, and 21 cases were found by physical examination. The preoperative diagnosis rate of CT and ultrasound was 40.2% (43/107) and 25.5% (24/94) respectively. The postoperative pathological types contained 83 cases of LAMN, 12 cases of mucinous adenocarcinoma, 9 cases of appendiceal neuroendocrine neoplasms (aNEN), 2 cases of mucinous adenocarcinoma with signet ring cells, 3 cases of serrated adenoma, 2 cases of goblet cell carcinoid, 2 cases of lymphoma, 1 case of leiomyoma and 1 case of schwannomas. All the patients underwent surgical resection, including 41 cases of appendectomy, 21 cases of partial cecectomy, 48 cases of right hemicolectomy, and 5 cases of combined organ resection due to appendiceal tumor infiltration or dissemination. Eighteen cases were diagnosed with pseudomyxoma peritonei (PMP) during operation. A total of 98 patients were enrolled for follow-up. The median follow-up time was 58 (5-172) months. The 5-year disease-free survival (DFS) rate was 84.5% and 5-year overall survival (OS) was 88.2%. Multivariate analysis revealed that high-grade malignancy tumors (HR=25.881, 95% CI: 2.827-236.935, P=0.004) and PMP formation (HR=42.166, 95% CI: 3.470-512.439,P=0.003) were independent risk factors for prognosis. Patients undergoing right hemicolectomy presented longer operation time, more blood loss and higher morbidity of complication as compared to those undergoing appendectomy and partial cecectomy (all P<0.05), while no significant differences in 5-year DFS (P=0.627) and 5-year OS (P=0.718) were found. Conclusions: Primary appendiceal neoplasms usually have no typical features, accompanied with low preoperative diagnosis rate. The common pathological types are LAMN, mucinous adenocarcinoma and aNEN. Appendectomy or partial cecectomy for LAMN may achieve satisfactory prognosis. High-grade malignancy tumors and PMP formation are independent risk factors for prognosis.
Adenocarcinoma, Mucinous/surgery* ; Appendectomy ; Appendiceal Neoplasms/surgery* ; Humans ; Male ; Neoplasm Recurrence, Local ; Retrospective Studies

Mucinous carcinoma (MC) is a rare subtype of breast cancer, which is composed of tumor cells floating in the abundant extracellular mucin. This form of cancer is usually estrogen receptor/progesterone receptor positive and human epidermal growth factor receptor 2 (HER2) negative. Here, we present a case of HER2-positive MC with an unusual signet ring cell differentiation. It is very rare that a breast tumor consists entirely of signet ring cells. The tumor showed pathologic complete response (pCR) after neoadjuvant chemotherapy with trastuzumab and pertuzumab. pCR of HER2-positive MC has rarely been described in literature. It is important to consider the biological heterogeneity of MCs for effective management.
Adenocarcinoma, Mucinous ; Breast Neoplasms ; Carcinoma, Signet Ring Cell ; Cell Differentiation ; Drug Therapy ; Epidermal Growth Factor ; Estrogens ; Humans ; Mucins ; Neoadjuvant Therapy ; Polymerase Chain Reaction ; Population Characteristics ; Receptor, Epidermal Growth Factor ; Receptor, ErbB-2 ; Trastuzumab