Endoscopic diagnosis and treatment of an appendiceal mucocele: A case report

BACKGROUNDAppendiceal mucocele is a rare disease that causes obstructive dilatation of the appendix due to the intraluminal accumulation of mucin. We report a case of endoscopic diagnosis and treatment of an appendiceal mucocele.CASE SUMMARYA 47-year-old man presented with a protrusion around the orifice of the appendix discovered by colonoscopy incidentally. He was admitted to our hospital for a routine checkup without any symptoms. Abdominal computed tomography showed a cystic mass approximately 3 cm in diameter with fat stranding. The preoperative diagnosis was non-neoplastic appendiceal mucocele, and endoscopic treatment was performed. The endoscopic findings and pathological results supported our preoperative diagnosis. The endoscopic treatment of appendiceal mucocele was feasible and effective, which was confirmed by repeated endoscopy and post-operative computed tomography after 7 mo. CONCLUSIONEndoscopic therapy provides a new method for the treatment of appendiceal mucocele.     

Treatment of a giant low-grade appendiceal mucinous neoplasm: A case report

BACKGROUNDLow-grade appendiceal mucinous neoplasm (LAMN) is extremely rare and easily misdiagnosed before surgery.CASE SUMMARYWe report the treatment of an asymptomatic case of LAMN diagnosed by magnetic resonance imaging (MRI) and surgical findings. A 70-year-old woman presented with an adnexal mass found by physical examination in July 2020. Gynecologic ultrasonography revealed a cystic mass in the right adnexa, and computed tomography showed a cystic mass in the pelvic cavity. All tumor markers were normal. A further MRI examination suggested mucinous neoplasm in the right pelvic cavity, excluding the possibility of adnexal cyst. Laparoscopic exploration found a huge cystic mass of about 10 cm × 7 cm that originated from the apex of the appendix, with spontaneous rupture. LAMN was confirmed by pathological examination. As of May 2021, no disease recurrence occurred after an open appendectomy.CONCLUSIONThis case indicates that we should pay more attention to female patients who are clinically diagnosed with an adnexal mass at admission,. The physical examination should be done carefully, and the laboratory and imaging examination results should be comprehensively analyzed to avoid misdiagnosis and to ensure prompt diagnosis and treatment, and to improve prognosis. MRI may be a better option for the diagnosis of appendiceal mucinous neoplasm.     

Mucinous cystic neoplasm of the liver: A case report

BACKGROUNDMucinous cystic neoplasm of the liver (MCN-L) is a cyst-forming epithelial neoplasm. The most distinguishing feature is the ovarian-type subepithelial stroma on pathological examination. CASE SUMMARYAn abdominal ultrasound incidentally revealed a liver tumor in a 32-year-old woman. Physical and laboratory examination results did not reveal any abnormalities. Enhanced abdominal computed tomography (CT) revealed a cystic space measuring 7.2 cm × 5.4 cm in the liver. Subsequent CT showed an increase in tumor size. Thus, we performed surgical resection of the tumor and gallbladder. Postoperative histopathological examination confirmed the diagnosis of MCN-L. At the 6-mo of follow-up, no recurrence was observed on ultrasound or CT.CONCLUSIONSince preoperative diagnosis of MCN-L is difficult, active surgery is recommended and helpful for the diagnosis and treatment of MCN-L.     

Low-grade mucinous appendiceal neoplasm mimicking an ovarian lesion: A case report and review of literature

BACKGROUNDAppendiceal tumors are rare lesions that may not be easily differentiated from primary ovarian lesions preoperatively, despite the use of advanced diagnostic methods by experienced clinicians.CASE SUMMARYA 59-year-old G2P2 woman, with chronic pelvic pain, underwent a pelvic ultrasound that revealed an adnexal mass measuring 58 mm × 34 mm × 36 mm, with irregular borders, heterogeneous echogenicity, no color Doppler vascularization and without acoustic shadowing. Normal ovarian tissue was visualized in contact with the lesion, and it was impossible to separate the lesion from the ovary by applying pressure with the ultrasound probe. Ascites, peritoneal metastases or other alterations were not observed. With the international ovarian tumor analysis ADNEX model, the lesion was classified as a malignant tumor (the risk of malignancy was 27.1%, corresponding to Ovarian-Adnexal Reporting Data System category 4). Magnetic resonance imaging confirmed the presence of a right adnexal mass, apparently an ovarian tumor measuring 65 mm × 35 mm, without signs of invasive or metastatic disease. During explorative laparotomy, normal morphology of the internal reproductive organs was noted. A solid mobile lesion involved the entire appendix. Appendectomy was performed. Inspection of the abdominal cavity revealed no signs of malignant dissemination. Histopathologically, the appendiceal lesion corresponded to a completely resected low-grade mucinous appendiceal neoplasm (LAMN).CONCLUSIONThe appropriate treatment and team of specialists who should provide health care to patients with seemingly adnexal lesions depend on the nature (benign vs malignant) and origin (gynecological vs nongynecological) of the lesion. Radiologists, gynecologists and other pelvic surgeons should be familiar with the imaging signs of LAMN whose clinical presentation is silent or nonspecific. The assistance of a consultant specializing in intestinal tumors is important support that gynecological surgeons can receive during the operation to offer the patient with intestinal pathology an optimal intervention.     

超声误诊阑尾低级别黏液性肿瘤1例

阑尾低级别黏液性肿瘤（Low- grade appendiceal mucinous neoplasm，LAMN）是阑尾黏液性肿瘤的一种少见类型，发生率占阑尾切除病例的0.2%~ 0.7%［1］，属于交界性或低度恶性肿瘤，破裂后易形成低级别腹膜假黏液瘤（PMP），临床症状个体差异大。LAMN起源于阑尾腺上皮，特点是由于粪石或者慢性炎症导致管腔狭窄，黏液物质聚积引起管腔扩张，黏液呈“推挤性”模式进入阑尾壁内或壁外［2］。超声表现：典型特征为“洋葱皮”样征象，大部分表现为囊实性包块，分隔纤细，部分病例内部回声呈胶冻状［3］，合并PMP时常伴有腹水。     

A case report of appendiceal adenocarcinoma without gastrointestinal evidence mimicking primary ovarian cancer

Metastatic tumours to the ovary comprise 10–25% of ovarian malignancies and may originate from various primary sites. Here, the case of a 49-year-old female patient who presented with periumbilical nodules and abdominal bloating is reported. She was found to have bilateral ovarian tumours with peritoneal carcinomatosis and ascites. Primary ovarian cancer was suspected while no contributory gastrointestinal lesion was detected by imaging studies and endoscopic examinations. Three cycles of neoadjuvant chemotherapy were administered, followed by interval debulking surgery. Appendiceal cancer was highly suspected based on analysis of a frozen section obtained during surgical debulking. Following the pathology investigation, the patient was finally diagnosed with primary appendiceal adenocarcinoma. She underwent chemotherapy comprising irinotecan and fluorouracil. Due to disease progression despite several chemotherapy regimens, the patient declined further treatment and was lost to follow-up 1 year after the debulking surgery. Metastatic tumours to the ovary may mimic primary ovarian cancers and often present with nonspecific manifestations. Therefore, meticulous exploration of the primary site is warranted if the diagnosis is clinically suspicious.     

Locally advanced perforated appendiceal cancer: Case report and review

Appendiceal cancers may be difficult to diagnose even after comprehensive investigation. This report of locally advanced perforated appendiceal adenocarcinoma attached to the terminal ileum, cecum, and rectosigmoid illustrates the management challenges that require comprehensive knowledge of pathologic variations and range from simple appendectomy to cytoreductive surgery and hyperthermic intraperitoneal chemotherapy.     

Malignant transformation of pulmonary bronchiolar adenoma into mucinous adenocarcinoma: A case report

BACKGROUNDBronchiolar adenoma (BA) and ciliated muconodular papillary tumor are rare tumors that have bilayered cell proliferation and continuous expression of p40 and CK5/6 in the basal cell layer. Diagnosis is difficult because of the limited knowledge of these tumors and their morphological similarities to malignant tumors, including invasive mucinous adenocarcinoma, especially based on the histopathology of intraoperative frozen sections. These tumors are now considered to be benign neoplasms, with malignant transformation reported in only a few cases. CASE SUMMARYA 57-year-old woman presented with a 17.0 mm × 7.0 mm nodule in the lower lobe of the left lung. Hematoxylin-eosin staining and immunohistochemistry of a surgical specimen were performed. The tumor consisted of a BA area and a mucinous adenocarcinoma (MA) area. In the BA area, the tumor had a bilayered structure of luminal cells and basal cells. The basal cells were positive for CK5/6 and p40, but the MA area was negative for these biomarkers. The Ki-67 proliferation index was low (1%-2%). The patient was diagnosed with BA accompanied by MA, and had a favorable outcome.CONCLUSIONThe present study indicated that BA may be carcinogenic, and suggests that clinicians should be aware of its potential for malignant transformation.     

Pseudomyxoma peritonei originating from intestinal duplication: A case report and review of the literature

BACKGROUNDPseudomyxoma peritonei (PMP) is a rare mucinous neoplasm with a relatively low incidence of 1 to 2 per million individuals. It is typically characterized by a type of gelatinous ascites named “jelly belly”. Most cases of PMP occur in association with ruptured primary mucinous tumors of the appendix (90%). Periodically, PMP can originate from mucinous carcinomas at other sites, including the colorectum, gallbladder, and pancreas. However, unusual origin can occur, as noted in this case report.CASE SUMMARYA 52-year-old woman had an unusual derivation of PMP from intestinal duplication. The patient complained of abdominal distension and increasing abdominal girth. Abdominal contrast-enhanced computed tomography showed a mass in the greater omentum located on the left side of the abdomen, likely to be a cystic mass of peritoneal origin. A PMP diagnosis was presumed based on the specific signs of the mass with flocculent and stripe-like echoes in ultrasound images. Ultrasound-guided percutaneous aspiration suggested a high likelihood of PMP. Once the PMP diagnosis was recognized, identification of the origin of the primary tumor was indicated. Thus, an exploratory laparoscopy was performed. In the absence of a primary tumor of appendix origin, the diagnosis of a low-grade mucinous neoplasm of intestinal duplication origin was finally confirmed by histopathology.CONCLUSIONPMP is secondary to mucinous carcinomas of the appendix mostly. This case resulted from an unusual derivation from intestinal duplication.     

Pancreatic cancer secondary to intraductal papillary mucinous neoplasm with collision between gastric cancer and B-cell lymphoma: A case report

BACKGROUNDPatients with intraductal papillary mucinous neoplasm (IPMN) have an increased risk of pancreatic and extrapancreatic malignancies. Lymphomas are rare extrapancreatic malignancies, and in situ collisions of early gastric cancer and diffuse large B-cell lymphoma (DLBCL) are even rarer. Here, we report the first case of pancreatic cancer comorbid with in situ collision of extrapancreatic malignancies (early gastric cancer and DLBCL) in a follow-up IPMN patient. Furthermore, we have made innovations in the treatment of such cases.CASE SUMMARYAn 81-year-old Japanese female diagnosed with IPMN developed elevated carbohydrate antigen (CA) 19-9 levels during follow-up. Because her CA19-9 levels continued to rise, endoscopic ultrasound (EUS) was performed and revealed a suspicious lesion at the pancreatic tail. However, lesions in the pancreas were not found by computed tomography, magnetic resonance imaging, or endoscopic retrograde cholangiopancreatography. To make an exact patho-logical diagnosis, EUS-guided fine needle aspiration was performed. To our supprise, early gastric cancer was found in preoperative gastroscopy. The gastric cancer was completely resected through endoscopic submucosal dissection before postoperative pathology identified early adenocarcinoma collided with DLBCL. Subsequent EUS-guided fine needle aspiration provided pathological support for the pancreatic cancer diagnosis, and then laparoscopic distal pancreatectomy and splenectomy were performed. CA19-9 levels returned to normal postoperatively. CONCLUSIONEndoscopic submucosal dissection is appropriate for submucosal lymphomas in patients intoleratant of chemotherapy. EUS can detect small IPMN-related pancreatic tumors.     

Primary maxillary chondrosarcoma: A case report

BACKGROUND Sarcomas of the head and neck region are rare tumors,constituting less than 1%of malignant neoplasms in this area,of which few cases(20%)originate from bone or cartilage.Chondrosarcoma is a malignant neoplasm that develops in bone,with a predilection for the pelvis,chest wall,and scapula,and is uncommon in the maxilla and jaw.Although this type of lesion has locally aggressive behavior,destroying the affected bone,it can metastasize when it is not diagnosed early and compromise the patient's life.CASE SUMMARY On intraoral examination of a 32-year-old female with a tumor in the middle third of the face,a well-defined rise in volume of approximately 3 cm in diameter was observed.Computed tomography with 3-dimensional reconstruction was performed,and we observed that the osteolytic lesion affected the vestibular cortex as the palatal bone.Hematoxylin and eosin staining revealed an appearance that was similar to mature hyaline cartilage,hypercellularity,nuclear and cellular pleomorphism,and multinucleated cells,with significant vacuolization.CONCLUSION Determination of the clinical and histopathological characteristics of rare neoplasms in the maxillofacial region,such as chondrosarcomas,allows the pathologist and surgeon to make the appropriate therapeutic decisions,optimizing the patient’s prognosis.     

Concomitant paraganglioma and thyroid carcinoma:A case report

BACKGROUND Paraganglioma/pheochromocytoma and medullary thyroid carcinoma can coexist and are often found in multiple endocrine neoplasia(MEN). However,very few cases highlight papillary thyroid carcinoma. We present herein a rare case of head and neck paraganglioma associated with papillary thyroid carcinoma.CASE SUMMARY A 51-year-old man presented to our department with right-sided neck swelling and hypertension. Physical examination showed neck masses with obvious pulsation. Concentrations of serum calcium, phosphorus, parathormone, thyroid stimulating hormone, free thyroxine, and calcitonin were within normal limits.Enhanced computed tomography revealed an irregular solid nodule, located in the carotid artery bifurcation. A low-density nodule of the thyroid isthmus with a spot-like dense shadow was also detected. The diagnosis of carotid body tumor was raised and an ultrasound-guided fine needle aspiration biopsy of the thyroid nodule revealed papillary thyroid carcinoma. The patient underwent surgery for lesion excision, total thyroidectomy, and neck dissection, and the pathology was reported as paraganglioma and papillary carcinoma. Genetic studies showed negative results for germline mutation of succinate dehydrogenase subunit D on 11q23. He was treated with ¹³¹I after surgery and remained disease-free so far.CONCLUSION The presence of concomitant paraganglioma and thyroid papillary carcinoma could be either coincidental or a result of an unknown mutation.     

Procedure of choice in a patient initially operated for a suspected epithelial appendiceal neoplasm

In patients operated for a suspected appendiceal neoplasm, radical appendectomy is the procedure of choice because it provides definitive treatment in most of appendiceal neoplasms, except from mucinous or colonic‐type adenocarcinoma and NET>2 cm.