Necrotising cellulitis of the breast associated with a fungating mucinous adenocarcinoma.

Breast necrotising soft tissue infections (NSTIs) are rare surgical emergencies with limited cases described in the literature. Here, we discuss a unique case of a woman in her 70s who presented with newly diagnosed diabetes and a neglected right breast cancer associated with breast erythema, skin necrosis, crepitus on examination and breast soft tissue gas seen on CT requiring emergent total mastectomy with partial pectoralis muscle excision. Pathology revealed a 15 cm invasive mucinous adenocarcinoma and necrotising polymicrobial cellulitis with a large abscess cavity. She recovered from her surgery with strict glycaemic control and a 10-day course of antibiotics. Multidisciplinary tumour board recommended adjuvant anastrozole, abemaciclib and postmastectomy radiation to complete her oncological treatment. Although exceedingly rare, it is important that clinicians be aware of, promptly recognise and properly treat NSTIs of the breast, as correct care can be life-saving from both infection and malignancy.

The relationship between observation interval and prognosis in pancreatic cancer concomitant with intraductal papillary mucinous neoplasia.

BACKGROUND: Intraductal papillary mucinous neoplasia (IPMN) is a risk factor for pancreatic cancer (PC). PC concomitant with IPMN shows rapid progression similar to de novo PC, therefore, the appropriate observation interval (OI) is not yet clear. PATIENTS AND METHOD: This was a multicenter retrospective observational study, and patients with PC concomitant with IPMN were analyzed. OI was defined as the interval between the date of imaging at PC diagnosis and just before the diagnosis. Clinical factors of PC and prognosis were assessed according to OI. RESULTS: From January 2010 to December 2018, 73 patients from 11 institutions were enrolled. The images performed just before PC diagnosis were contrast-enhanced CT/magnetic resonance imaging/endoscopic ultrasonography in 44/27/2 patients, respectively. The median cyst size was 14.0 mm, and the median main pancreatic duct diameter was 3.0 mm. The median OI was 6.8 months. In OI 6 months or less (OI ≤ 6 M)/OI more than 6 months (OI > 6 M), the mean tumor size, the frequencies of metastatic PC, resectable PC and early-stage PC were 20.1/21.5 mm (P = 0.91), 12.1 %/32.5 % (P = 0.05), 72.7 %/52.5 % (P = 0.09) and 27.3 %/25.0 % (P = 1.00), respectively. The median overall survival was 35.5 months in OI ≤ 6 M and 16.2 months in OI > 6 M (P = 0.05). CONCLUSION: In OI 6 months or less, the rate of resectable PC was high, however, the rate of early PC was almost the same as that of OI more than 6 months. Approximately 10 % of cases found in the advanced stage with metastasis even if OI 6 months or less.

Acute pancreatitis in intraductal papillary mucinous neoplasm: a single-center retrospective cohort study with systematic review and meta-analysis.

BACKGROUND: Intraductal papillary mucinous neoplasm (IPMN) is a cystic tumor of the pancreas arising from abnormal papillary proliferation of ductal epithelial cells, and is a precancerous lesion of pancreatic malignancy. This study aimed to evaluate associations between acute pancreatitis (AP) and histologic subtypes of IPMN. METHODS: In the clinical study, patients with IPMN confirmed by surgical resection specimens at our institute between 2009 and 2021 were eligible for inclusion. Associations and predictive accuracy of AP on the presence of HGD were determined by logistic regressions. In addition, a systematic review and meta-analysis was conducted through literatures upon search in PubMed, Embase, CENTRAL, China National Knowledge Infrastructure (CKNI), and Wanfang database, up to June, 2023. Pooled effects of the associations between AP and HGD and intestinal epithelial subtype subtype, shown as odds ratios (ORs) with 95% confidence intervals (CIs), were calculated using random effects model. RESULTS: The retrospective cohort study included 47 patients (32 males, 15 females) diagnosed with IPMN at our center between 2009 and 2021, including 11 cases with AP (median 62 years) and 36 cases (median 64.5 years) without. Accuracy, sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of AP in predicting HGD were 78.7%, 57.1%, 82.5%, 36.4%, and 91.7%, respectively. Univariate logistic regression analysis showed that AP group had greater odds of presence of HGD (OR: 6.29,95% CI: 1.14-34.57) than non-AP group. Meta-analysis of five case-control studies in the literature included 930 patients and showed that AP-IPMN patients had higher odds for HGD (OR: 2.13, 95% CI 1.38-3.29) and intestinal epithelial subtype (OR: 5.38, 95% CI: 3.50-8.27) compared to non-AP IPMN. CONCLUSIONS: AP is predictive of malignancy in patients with IPMN.

Cecal-Colon Intussusception due to Appendiceal Mucinous Adenocarcinoma.

Intussusception is a rare presentation in adults and describes when one portion of the intestine telescopes into another portion. Intussusception is associated with malignancies serving as the lead point in adults. Appendiceal mucinous neoplasms are uncommon tumors often incidentally discovered during appendectomy procedures to manage acute appendicitis. Here we present a case report of an instance of mucinous adenocarcinoma of the appendix that manifested as a large bowel obstruction with intussusception limited to the colon, underscoring the possibility of concurrent intussusception and mucinous neoplasms. The case highlights the importance of meticulous diagnostic evaluation and management, particularly without well-defined treatment protocols. Appropriate diagnostic workup and management, including surgical intervention, are critical for patient outcomes and overall prognosis. The study recommends that patients diagnosed with confirmed or suspected appendiceal neoplasms undergo upfront oncologic resection where aggressive malignancy is a concern. Colonoscopy should be performed postoperatively for all patients to identify synchronous lesions.

Gastric-phenotype Mucinous Carcinoma of the Fallopian Tube with Secondary Ovarian Involvement in a Woman with Peutz-Jeghers Syndrome: A Case Report.

Peutz-Jeghers syndrome is an autosomal dominant condition characterized by the association of hamartomatous polyps in the digestive tract, mucocutaneous pigmentation, family history, and infrequently tumors of the female genital tract with one of the most characteristic being the gastric-type endocervical adenocarcinoma. We present the case of a 75-year-old woman with a history of gastrointestinal polyps and cancer of the pancreas and breast, diagnosed with Peutz-Jeghers syndrome, who clinically debuted with a primary adnexal tumor. However, on histologic examination it was found to be a gastric-phenotype primary mucinous carcinoma tubal in origin, associated to tubal mucinous metaplasia and secondary ovarian involvement. One of her daughters had a confirmed genetic diagnosis of Peutz-Jeghers syndrome and presented with mucinous metaplasia of the tubal mucosa in the pathological study of a prophylactic hysterectomy specimen. Another of her daughters died from an ovarian juvenile granulosa cell tumor, she did not have a genetic diagnosis of Peutz-Jeghers syndrome. This case intends to highlight the rarity of gastrointestinal-type mucinous carcinomas of the ovary and fallopian tube (similar to gastric-type endocervical adenocarcinoma) in Peutz-Jeghers syndrome and emphasize the importance of genetic counseling of these patients as well as the adequate sampling of surgical specimens for early detection and treatment.

Gastric-type mucinous adenocarcinoma of the cervix in a woman with Peutz-Jeghers syndrome: a case report.

BACKGROUND: Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant disorder characterized by a predisposition to the development of multiple neoplasms. Gastric-type mucinous adenocarcinoma (GAS), a new variant of carcinoma of the cervix according to 2014 WHO classification, is less common compared with squamous cell carcinoma, is more aggressive and has a lower 5-year survival rate compared with the usual-type endocervical adenocarcinoma, and is also unrelated to human papillomavirus (HPV) infection. CASE SUMMARY: We herein present the case of a 32-year-old patient with PJS who was diagnosed with GAS of the cervix. The patient was not sexually active and had been diagnosed with PJS at 2 years of age. A tumor ∼6 cm was found on the cervix and was diagnosed as GAS of the cervix of clinical-stage IB3. The patient was treated with intra-arterial chemotherapy for one course, followed by radical surgery and then systematic chemotherapy. CONCLUSION: The present case highlights the need for more thorough cancer screening for patients with PJS, as this disorder is rare and is associated with a high risk of malignancies. Young patients with PJS, including those who are not sexually active, who present with watery vaginal discharge or bleeding should be screened for cervical carcinoma, even if the cytological results or HPV tests are negative.

Invasive Mucinous Adenocarcinoma with Acute Respiratory Distress Syndrome Diagnosed by Autopsy Findings.

We herein report a fatal case of invasive mucinous adenocarcinoma (IMA) with acute respiratory distress syndrome (ARDS) diagnosed based on autopsy findings. A 76-year-old man presented with severe respiratory discomfort on admission. Computed tomography (CT) revealed a peripheral distribution of consolidation. Although his respiratory status improved after steroid therapy, re-exacerbation occurred, and the patient died on day 131. A bronchoscopic lung biopsy had shown organizing pneumonia, but a post-mortem examination surprisingly revealed IMA with organizing pneumonia. IMA presenting with ARDS as the first symptom is extremely rare.

[A Case of Intraductal Papillary Mucinous Adenocarcinoma with Hepatic Dysfunction Due to Tumor Perforation into the Bile Duct].

A 58-year-old woman presented with a complaint of weight loss. Abdominal computed tomography showed dilatation of the biliary and pancreatic ducts and a mural nodule in the pancreatic duct. The diagnosis was intraductal papillary mucinous neoplasm(IPMN). Endoscopic retrograde cholangiopancreatography(ERCP)and cholangioscopy revealed a fistula between the common bile duct and the IPMN. A sudden increase in hepatobiliary enzymes was noted preoperatively. ERCP showed that the common bile duct was obstructed by mucus. A nasobiliary drainage tube was inserted into the bile duct endoscopically and kept open by daily tube washing, and the liver dysfunction improved. Total pancreatectomy, splenectomy, and regional lymph node dissection were performed. Histological examination confirmed that the primary tumor was mixed invasive intraductal papillary mucinous adenocarcinoma. The patient remains alive and well with no evidence of recurrence 18 months after resection.

Adult Colo-Colonic Intussusception in the Setting of Invasive Mucinous Adenocarcinoma: A Case Report.

BACKGROUND Colonic intussusception is a very rare disease in adults, and if present, is usually manifested by another pathology, such as malignancy. This report describes the diagnosis and treatment of the underlying cause of intussusception, which was spontaneously reduced. CASE REPORT A 39-year-old woman with no significant past medical history presented to St. Joseph's University Medical Center on July 2022 with gradually worsening abdominal pains for 1 year and hematochezia for 3 months. Physical examination was positive for left lower quadrant abdominal tenderness to palpation. A computed tomography scan of the abdomen and pelvis without contrast showed a long segment of intussusception involving the sigmoid colon and rectum, without any noticeable lesions. A repeat computed tomography scan with rectal contrast showed a 2.1×1.1-cm mesenteric mass in the sigmoid colon at the region of the intussusception. The patient was taken for a laparoscopic sigmoid resection with primary anastomosis, showing a 5-cm mass in the sigmoid colon, and surgical pathology confirming neoplastic etiology of intussusception. The patient recovered well after surgery, and was referred for oncological intervention soon afterward. CONCLUSIONS This report displays the importance of the type of imaging modalities with and without contrast to diagnosis and determine underlying causes of intussusception and further guide treatment options.

Long-standing diabetes mellitus increases concomitant pancreatic cancer risk in patients with intraductal papillary mucinous neoplasms.

BACKGROUND: When monitoring patients with an intraductal papillary mucinous neoplasm (IPMN), it is important to consider both IPMN-derived carcinoma and concomitant ductal adenocarcinoma (PDAC). The latter is thought to have a poorer prognosis. We retrospectively analyzed the risk factors for concomitant PDAC in IPMN. METHODS: In total, 547 patients with pancreatic cysts, including IPMNs inappropriate for surgery on initial diagnosis, encountered from April 2005 to June 2019, were reviewed. We performed surveillance by imaging examination once or twice a year. RESULTS: Five IPMNs with high-grade dysplasia and one IPMN associated with invasive carcinoma were encountered. In comparison, 14 concomitant PDACs were encountered. The prognosis was very poor for concomitant PDACs. All 14 PDAC patients had IPMNs. In patients with IPMNs, long-standing diabetes mellitus was the only significant risk factor for concomitant PDAC in both univariate and multivariate analyses (P < 0.001 and P < 0.01, respectively). Furthermore, patients with IPMNs and diabetes mellitus had a high frequency of concomitant PDACs (9.5%, 9/95) in a median 48-month surveillance period. CONCLUSIONS: When monitoring IPMNs, the development of not only IPMN-derived carcinomas but also concomitant PDACs should be considered. During this period, it may be prudent to concentrate on patients with other risk factors for PDAC, such as long-standing diabetes mellitus.

Pancreatic Adenocarcinoma Associated to Intraductal Papillary Mucinous Neoplasia: Histopathological Particularities and Clinical Implications.

Background: Pancreatic ductal adenocarcinoma (PDAC) is the most common pancreatic tumor, known for an aggressive evolution. Intraductal papillary mucinous neoplasm (IPMN) is a rare pancreatic tumor, considered a premalignant lesion with the possibility of carcinogenesis towards PDAC. The clinical, surgical and histopathological particularities of the association between PDAC and IPMN are yet unknown, further research being needed. Methods: We have conducted a retrospective descriptive study, on a nine-year period (2012-2020), with the aim of comparing the characteristics of patients that underwent curative surgical interventions for solitary PDAC and PDAC associated to IPMN. Results: Fifteen patients with PDAC associated with IPMN (Group 1) and 386 patients with solitary PDAC (Group 2) were included in our study. Group 1 had a younger average age (61.8 years) compared to Group 2 (63.89 years). Total pancreatectomy was more frequently performed for Group 1 than Group 2 (33.33% vs 12.43%). Group 1 had a higher percentage of cases with positive perineural, perilymphatic and perivascular invasion. Group 1 registered a worse overall survival, as well as a worse short-time survival compared to Group 2. Conclusions: PDAC associated to IPMN registers distinct epidemiological, clinical and histopathological characteristics compared to solitary PDAC.

Mucinous adenocarcinoma of a tailgut cyst.

A tailgut cyst is a rare congenital lesion that develops from a residual posterior remnant of the intestine and presents as a mass in the presacral space. They are generally asymptomatic or have atypical symptoms, are usually benign but may rarely become malignant. We report a case of a 37-year-old female who initially presented to the Surgical Department of Korle Bu Teaching Hospital, Accra, Ghana with a malignant tailgut cyst after having repeated surgical procedures for recurrent perianal infective pathologies but still had persistence of symptoms. The lesion was initially excised and found to be a dermoid cyst histologically. The mass recurred after a year and had a re-excision; the lesion was diagnosed histologically as mucinous adenocarcinoma. This report emphasizes the different forms of presentation of a patient with a tailgut cyst and the possibility of malignant transformation, as well as the presentation of this tailgut cyst which can be diagnosed using radiological investigations and histological findings. Funding: None.

Absceso del psoas ilíaco secundario a un tumor de colon izquierdo fistulizado al retroperitoneo: caso clínico y análisis de la literatura / Iliopsoas abscess resulting from penetrating left colon cancer to the retroperitoneum: case report and literature review / Abscesso do iliopsoas secundário a tumor de cólon esquerdo fistulizado para o retroperitônio: caso clínico e análise da literatura

Los abscesos del psoas ilíaco secundarios a un tumor de colon fistulizado son excepcionales y potencialmente graves. La mayoría son adenocarcinomas de tipo mucinoso. Su tratamiento es complejo ya que, para lograr una resección oncológica pretendidamente curativa, es necesario realizar una resección ampliada con mayor morbimortalidad. Presentamos el caso de una paciente con un adenocarcinoma mucinoso de colon izquierdo fistulizado al músculo ilíaco y la pared anterolateral del abdomen en la que se realizó una resección multivisceral que incluyó el colon izquierdo, el músculo y la cresta ilíaca y parte de la pared anterolateral del abdomen.

Iliopsoas abscess secondary to perforation of colon cancer is an extremely rare and potentially life-threatening condition. Most tumors are mucinous adenocarcinomas. Its treatment its complex, as most patients need radical extended resections to achieve good oncological results, which are in turn, graved with higher morbidity and mortality. We present the case of a patient with a left colon mucinous adenocarcinoma penetrating to the iliopsoas muscle and the anterolateral abdominal wall that required a multivisceral resection including left colon, iliac muscle and crest and part of the anterolateral abdominal wall.

Abscessos do iliopsoas secundários a um tumor de cólon fistulizado são raros e potencialmente graves. A maioria são adenocarcinomas do tipo mucinoso. Seu tratamento é complexo, pois, para se obter uma ressecção oncológica supostamente curativa, é necessário realizar uma ressecção ampliada com maior morbimortalidade. Apresentamos o caso de um paciente com adenocarcinoma mucinoso de cólon esquerdo fistulizado para o músculo ilíaco e parede ântero-lateral do abdome no qual foi realizada ressecção multivisceral que incluiu cólon esquerdo, músculo e crista ilíaca e parte do a parede anterolateral do abdome.

A case of juvenile systemic sclerosis and congenital pulmonary airway malformation related mucinous adenocarcinoma of the lung: paraneoplastic syndrome or just a coincidence?

BACKGROUND: Juvenile systemic sclerosis (JSS) is an extremely rarely seen auto-immune disease characterized by the increased fibrosis of skin and internal organs. Congenital pulmonary airway malformation (CPAM) is a developmental disorder of the lung, characterized by atypical cell hyperplasia which creates the ground for lung adenocarcinoma. In general, CPAM is diagnosed in early childhood, due to recurrent respiratory symptoms including cough, hemoptysis and respiratory infections. Although rare, there are some sporadic asymptomatic cases of CPAM that have been reported. We present a case with a coincidental presence of two rare diseases: JSS and CPAM. CASE: An adolescent female patient was admitted to hospital due to clinical signs of JSS. During the followup, the patient had been diagnosed with cystic adenoid malformation of the lung complicated by mucinous adenocarcinoma. The patient was previously healthy with an unremarkable history, including lack of respiratory symptoms. Left inferior lobectomy was performed. Considering the small size of malignant loci, the total resection of the tumor and absence of any sign for metastasis disease, adjuvant therapy was not scheduled. We haven`t found a pediatric case of CPAM associated adenocarcinoma of the lung presented by signs of JSS in the literature. In this case, the clinical signs of JSS possibly represent part of the paraneoplastic syndrome related to adenocarcinoma of the lung. CONCLUSIONS: Internal organ involvement, including respiratory system, should not be omitted even in asymptomatic patients with JSS. Auto-antibody negativity represents a clue for the possible underlying condition. Further studies with a higher number of patients would reveal more relevant data.

[A Case Report of Subcutaneous Nodular Fat Necrosis Caused by Obstructive Pancreatitis Due to IPMN].

A 72-year-old man underwent hemodialysis because of end-stage renal failure. The patient often suffered acute pancreatitis and was diagnosed with main duct type intraductal papillary mucinous neoplasm(IPMN). Moreover, skin erythema with pain occurred and was treated as cellulitis using antibiotics; however, the skin lesions did not improve. Skin pathological findings indicated subcutaneous nodular fat necrosis due to pancreatitis. Subtotal stomach-preserving pancreaticoduodenectomy was performed, and the skin erythema with pain symptoms were relieved. The final diagnoses were ampullary carcinoma and intraductal papillary mucinous adenoma(IPMA). We experienced a rare case of subcutaneous nodular fat necrosis due to IPMN.

[A Rapid Clinical Course of Pulmonary Embolism Associated with Gallbladder Mucinous Carcinoma].

A 51-year-old woman with edema of the lower extremities and exertional dyspnea was admitted to our hospital. Enhanced CT revealed thrombi of the pulmonary artery and a gallbladder tumor. After anticoagulation therapy was started on her, anemia and jaundice progressed; thus, endoscopic retrograde cholangiopancreatography(ERCP)was performed on suspicion of bleeding from a gallbladder tumor. We performed cholecystectomy in emergency to control the anemia due to hemorrhage. Oxygenation suddenly worsened intraoperatively, maintaining her blood pressure became difficult, and the patient decompensated. The histopathological diagnosis was gallbladder mucinous carcinoma with severe lymphatic invasion. Although an autopsy was not performed, pulmonary artery embolism derived from a tumor embolus was the suspected cause of the sudden change of the clinical course.

Ovarian Clear Cell Tumors Associated With Seromucinous Borderline Tumor: A Case Series.

Ovarian seromucinous borderline tumors (SMBT) and clear cell tumors are both closely associated with endometriosis and share, in a proportion of cases, a molecular pathway involving ARID1A mutations, but they have been rarely described in association. We report a case series of 4 clear cell tumors (3 carcinomas, 1 borderline adenofibroma) coexisting in the same ovary with SMBT. In all cases, the SMBT was the predominant component and we highlight that adequate sampling of these tumors is important to detect small clear cell carcinomas, thus potentially altering the treatment and prognosis.

Xanthogranulomatous Pancreatitis Accompanied by Xanthogranulomatous Cholecystitis: A Case Report and Literature Review.

ABSTRACT: Xanthogranulomatous pancreatitis (XGP) is extremely rare, with only 31 cases reported in the English literature to date. We reviewed previously reported 17 articles about XGP and report an additional case of XGP. This is the first case of XGP with xanthogranulomatous cholecystitis accompanied by intraductal papillary mucinous carcinoma (IPMC) in an 80-year-old woman. She was referred to our hospital with jaundice and general malaise and was found to have a cystic mass at the pancreatic head and a solid mass at the pancreatic tail, with dilation of both the main pancreatic duct and biliary tract noted on computed tomography. Diagnosis of IPMC at the pancreatic head with neuroendocrine tumor at the pancreatic tail was made, and the patient underwent subtotal stomach-preserving pancreatoduodenectomy with enucleation of the mass at the tail. Pathological examination revealed IPMC with xanthogranulomatous changes around the IPMC and at the pancreatic tail and gallbladder. Xanthogranulomatous pancreatitis could be induced by inflammatory reaction due to obstruction of the pancreatic duct and biliary tract by mucin produced in the IPMC. It is sometimes difficult to preoperatively differentiate XGP from malignant pancreatic tumors, such as pancreatic carcinoma or neuroendocrine tumor, using imaging studies.