Dermoscopy of Umbilical Lesions-A Systematic Review.

Background: The umbilicus is a fibrous remnant located in the centre of the abdomen. Various entities may be encountered in this special anatomical location; however, little is known about their dermoscopic presentation. The aim of this study was to provide a comprehensive summary of existing evidence on dermoscopic features of umbilical lesions. Methods: Studies assessing dermoscopic images of umbilical lesions were included in this study. No age, ethnicity or skin phototype restrictions were applied. Papers assessing lesions outside of the umbilical area, lacking dermoscopic images and/or dermoscopic description and not related to the topic were excluded. Embase, Medline and Cochrane Library were searched from inception to the end of May 2023. The Joanna Briggs Institute critical appraisal tools were used to evaluate the risk of bias of the selected studies. The quality and the level of evidence of included studies were assessed according to the Oxford 2011 Levels of Evidence. Thirty-four studies reporting a total of 39 lesions met the inclusion criteria and were included in qualitative analysis. Results: A qualitative synthesis of the following entities was performed: melanoma, nevi, basal cell carcinoma, fibroepithelioma of Pinkus, Sister Mary Joseph nodule, mycosis fungoides, dermatofibroma, endometriosis, epidermal cyst, granuloma, intravascular papillary endothelial hyperplasia, lichen planus, omphalolith, seborrheic keratosis, and syringoma. Conclusions: Dermoscopy is a non-invasive technique that may be useful in the differential diagnosis of umbilical lesions. The main limitations of this study were lack of a high level of evidence in the studies and the lack of uniformity in applied dermoscopic terminology between included studies.

Long-term survival of the Sister Mary Joseph nodule originating from breast cancer: A case report.

BACKGROUND: A Sister Mary Joseph nodule (SMJN) is an uncommon cutaneous metastasis found in the umbilicus, indicating an advanced malignancy. SMJNs typically originate from intra-abdominal sources, rarely from breast cancer. Diagnosis suggests a poor prognosis with a median survival of approximately 8 mo after detection. Managing patients with SMJNs is challenging, as most receive limited palliative care only. The optimal strategy for long-term survival of these patients remains unclear. CASE SUMMARY: A 58-year-old female, previously diagnosed with right breast cancer 17 years ago and underwent breast-conserving surgery, adjuvant radiotherapy, and endocrine therapy, presented with a 2-cm umbilical nodule. Thirteen years previously, metastases were detected in the right supraclavicular, infraclavicular, hilar, and mediastinal lymph nodes. An umbilical nodule emerged four years before the date of presentation, confirmed as a skin metastasis of primary breast cancer upon excisional biopsy. Despite initial removal, the nodule recurred and grew, leading to her referral to our hospital. The patient underwent extensive excision of the umbilical tumor and immediate abdominal wall reconstruction. Endocrine therapy was continued postoperatively. Five years later, no local recurrence was observed, and the patient continued to work full-time, achieving over 9 years of survival following SMJN diagnosis. CONCLUSION: This case study aimed to identify the optimal strategy for achieving extended survival outcomes in patients with SMJN through comprehensive treatment. We presented a case of the longest survival in a patient after undergoing a multidisciplinary treatment regimen. Our findings underscore the significance of adopting a multimodal treatment approach comprising timely and wide excision along with adjunctive therapy. This approach can control the disease, prolong survival, and improve the quality of life in patients with SMJN.

Diffuse peritoneal carcinomatosis and Sister Mary Joseph nodule in ovarian carcinoma - exquisite demonstration of the peritoneal reflections on [18F]FDG PET/CT.

A case involving a 64-year-old woman with ovarian carcinoma on maintenance therapy who underwent 18-fluorodeoxyglucose positron emission tomography with computed tomography ([18F]FDG PET/CT) restaging due to rapid cancer antigen 125 (Ca-125) rise. This revealed recurrent disease within the pelvis and large volume, peritoneal carcinomatosis including an avid umbilical deposit, consistent with the rarely seen Sister Mary Joseph nodule (SMJN). This case elegantly demonstrates not only the anatomy of the peritoneal surfaces through avid disease deposition but also highlights the sensitive depiction of disease burden in peritoneal carcinomatosis, including the detection of rare manifestations such as SMJN.

Dedifferentiated umbilical metastases from low grade endometrioid endometrial adenocarcinoma complicated by super-obesity: A case report.

INTRODUCTION: Umbilical metastases are uncommon and rarely associated with endometrial cancer. In this report we describe a unique case of a patient with low-grade endometrioid adenocarcinoma (EAC) who developed an umbilical metastasis containing dedifferentiated endometrial carcinoma, in the context of super-morbid obesity with a body mass index (BMI) of 80 kg/m2. PRESENTATION OF CASE: A 55-year-old female with atypical endometrial hyperplasia was treated with progestogens whilst attempting weight loss prior to definitive surgery, given the impact of her obesity on surgical risk. She progressed to grade 1 EAC of the uterus and then to metastatic disease in the umbilicus and inguinal lymph nodes. After adequate weight loss, cytoreductive surgery was completed via robotic hysterectomy and bilateral salpingoophorectomy (BSO) and resection of metastatic disease. Differing histological grades were seen across all disease sites with dedifferentiated endometrial carcinoma at the umbilicus. Immunohistochemistry suggested a common uterine origin due to loss of MLH1 and PMS2 proteins. DISCUSSION: There are no clear management pathways for the rare umbilical metastases related to endometrial cancer. Our patient's significant obesity complicated their clinical course and highlights the risks of progestogen therapy whilst attempting weight loss prior to definitive surgery for low-grade EAC. Differing grades of disease across metastatic sites in EAC is rare and may reflect the recently recognized intratumoral genetic heterogeneity in endometrial cancers. CONCLUSION: Management of umbilical metastases should be individualized to patient factors and local resources. More research is needed into pathways of care for women with low grade EAC with obesity preventing routine surgical management.

Cholangiocarcinoma Resembling IgG4-related Sclerosing Cholangitis.

A 66-year-old man diagnosed with immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) with diffuse intrahepatic bile duct stenosis and elevated serum IgG4 levels was referred for a further examination because of elevated serum carbohydrate antigen 19-9 levels despite treatment with corticosteroids. An umbilical nodule was found on a physical examination and a biopsy showed adenocarcinoma. Although several imaging studies revealed no changes from prior studies, bile cytology collected by endoscopic retrograde cholangiopancreatography showed adenocarcinoma. Consequently, the patient was diagnosed with cholangiocarcinoma resembling IgG4-SC after detecting an umbilical metastasis, also known as Sister Mary Joseph's nodule.

Pancreatic cancer: Cutaneous metastases, clinical descriptors and outcomes.

BACKGROUND: Cutaneous metastases in pancreatic cancer (PC) are rare. Herein, we evaluate the clinical, genomic, and other descriptors of patients with PC and cutaneous metastases. METHODS: Institutional databases were queried, and clinical history, demographics, PC cutaneous metastasis details, and overall survival (OS) from cutaneous metastasis diagnosis were abstracted. OS was estimated using Kaplan-Meier methods. RESULTS: Forty patients were identified, and median age (Q1-Q3, IQR) of PC diagnosis was 66.0 (59.3-72.3, 12.9) years. Most patients had Stage IV disease at diagnosis (n = 26, 65%). The most common location of the primary tumor was the tail of the pancreas (n = 17, 43%). The most common cutaneous metastasis site was the abdomen (n = 31, 78%), with umbilical lesions occurring in 74% (n = 23) of abdominal lesions. The median OS (95% CI) was 11.4 months (7.0, 20.4). Twenty-three patients had umbilical metastases (58%), and 17 patients had non-umbilical metastases (43%). The median OS (95% CI) was 13.7 (7.0, 28.7) months in patients with umbilical metastases and 8.9 (4.1, Not reached) months in patients with non-umbilical metastases (p = 0.1). Sixteen of 40 (40%) patients underwent somatic testing, and findings were consistent with known profiles. Germline testing in 12 (30%) patients identified pathogenic variants in patients: CHEK2, BRCA1, and ATM. CONCLUSION: Cutaneous metastases from PC most frequently arise from a pancreas tail primary site and most frequently occur in the umbilicus. Cutaneous metastases may generally be categorized as umbilical or non-umbilical metastases.

Sister Mary Joseph's Nodule From Renal Cell Carcinoma: A Case Report.

Sister Mary Joseph's nodules (SMJNs) are umbilical skin metastases of various abdominopelvic malignancies, and they rarely originate from renal cell carcinomas. Radiotherapy is typically used to treat the nodules as a palliative intention. We report a rare case of SMJN that originated from clear cell renal cell carcinoma, which was treated with external beam radiation therapy (EBRT) and interstitial brachytherapy (ISBT). A 74-year-old male patient with a history of left renal cell carcinoma developed an umbilical nodule which was diagnosed as SMJN. The patient underwent EBRT (30 Gy in 10 fractions) and ISBT (12 Gy in two fractions), leading the nodule to complete resolution. This case report might support that radiotherapy, including ISBT, is effective for the treatment of SMJN from renal cell carcinoma.

Metastatic Pancreatic Adenocarcinoma to Umbilical Skin.

Sister Mary Joseph nodule (SMJN) is a rare metastasis to umbilical skin originating from internal tumors including the stomach, ovary and large intestine and less commonly from pancreatic cancers. We report an uncommon case of metastatic pancreatic adenocarcinoma to umbilical skin. An 85-year-old female presented with a 1.8 cm protrusion of the right lateral umbilicus. The CT scan showed a 3.5 cm pancreatic lesion, peritoneal carcinomatosis and abdominal lymphadenopathy. Histology examination revealed atypical infiltrative glandular structures. Immunohistochemistry showed positive CK7, negative CDX2 and P53 with mutated patterns. These were consistent with metastatic adenocarcinoma most consistent with pancreatobiliary or upper GI origin. CK7 expresses in the ductal cells in pancreatic ductal adenocarcinoma. While CDX2 is positive in intestinal-type adenocarcinoma, it is negative in pancreatic ductal adenocarcinoma. The diagnosis of adenocarcinoma is rendered based on the presence of a pancreatic lesion in CT scan, positive CK7 and negative CDX2 in umbilical nodule tumor cells in the current patient.

Eponyms in medical oncology.

Eponyms have historically been used to honor individual contributions or discoveries in the field of medicine. More recently, some eponyms have been criticized for imprecision or for being misnomers. Eponyms attributed to discoveries made by Nazi German scientists have also fallen out of favor. However, despite these criticisms, eponyms remain popular for their ease of use. Eponyms generate interest in medical history and may help humanize the study of medicine. Here, we describe several eponyms in medical oncology with a focus on basic disease pathophysiology, epidemiology, and brief background on the individuals for whom the eponym was named.

Intravascular papillary endothelial hyperplasia mimicking sister Mary Joseph nodule in a patient with ovarian carcinoma

@#Intravascular Papillary Endothelial Hyperplasia (IPEH) is a rare benign vascular lesion of the skin and subcutaneous tissues that results from the proliferation of endothelial cells within a blood vessel. In this article, we present the case of an IPEH mimicking a Sister Mary Joseph Nodule on the umbilicus of a 53-year old female patient with ovarian cancer. A diagnostic workup was performed with a computerized tomography of abdominal cavity and pelvis showing an expansive cystic tumor formation with probable ovarian origin. Two separate histopathologic readings were done on the cutaneous lesion which revealed contradicting findings of benign versus malignant tumors. Immunohistochemical stains done showed that the lesion was positive for ERG and negative for epithelial differentiation markers. No surgical intervention was done at the time of consultation as a cutaneous metastasis was primarily considered initially. It is crucial to rule out a diagnosis of Sister Mary Joseph nodule, especially in a background of ovarian carcinoma as it may mimic vascular lesions occurring on the umbilicus. Immunohistochemical staining is a significant tool to precisely diagnose such lesions so that it is neither inadequately nor aggressively managed.

Sister Mary Joseph's nodule revealing a jejunal adenocarcinoma: A case report.

INTRODUCTION AND IMPORTANCE: Adenocarcinoma of the jejunum is a diagnostic challenge for the physician because of its extreme rareness and the fact that it classically presents with vague clinical symptoms. On the other hand, the Sister Mary Joseph's nodule is a rare clinical sign that refers to umbilical metastasis of an internal malignancy. We here report a rare case of jejunal adenocarcinoma revealed by a Sister Mary Joseph's nodule. CASE PRESENTATION: A 55-year-old man presented with an ulcerated umbilical tumor, which was found to be a secondary lesion of an advanced jejunal adenocarcinoma invading the transverse colon. He underwent surgical resection of the umbilical tumor and the intestinal primitive. CLINICAL DISCUSSION: The presence of umbilical metastasis usually represents advanced disease but can be its first manifestation. Gastro-intestinal tract tumors such as jejunal adenocarcinomas and gynecologic malignancies are the most common primary sites. CONCLUSION: Practicians must be aware of clinical implication of Sister Mary Joseph nodule. Aggressive surgery when feasible can be beneficial for survival.

Radical treatment of Sister Mary-Joseph nodule: case report and literature review.

Sister Mary-Joseph nodule (NSMJ) is a cutaneous metastasis of the umbilicus, rare and accounts for 2-3% of the patients with advanced stages of colorectal adenocarcinoma. Here we report the observation of a 48-year-old Moroccan man, referred to our hospital to manage a painful ulcero-budding nodule of the umbilicus; computed tomography revealed that the processes infiltrated the urachus and the bladder. Laboratory parameters were normal and radical surgery was performed to remove the tumor and embryological remnant of the umbilicus. The histological assessment confirmed the sigmoidal origin of the umbilical nodule. This kind of disease always poses a problem of treatment. It was considered for a long time as an outdated stage of tumor disease that deserves just palliative treatment. Several cases published in the international literature with radical treatment had good survival and evolution, which gives hope to patients with this disease.

18F-Fluorodeoxyglucose Positron-Emission Tomography/Computed Tomography Detected Inguinal Lymph Nodal Metastasis from Hepatocellular Carcinoma.

Hepatocellular carcinoma (HCC) usually metastasizes to the regional abdominal lymph node, lungs, and bones. Nonregional lymph node involvement by HCC in the absence of regional lymph nodes is rare. We describe the 18F-fluorodeoxyglucose positron-emission tomography-computed tomography findings of an HCC patient with histopathologically proven inguinal lymph nodal metastasis in the absence of regional lymph nodal metastases.

Metastasis of missed cholangiocarcinoma in the left lobe through abdominal wall laparoscopic port-site and umbilicus after laparoscopic cholecystectomy: Case report and literature review.

Laparoscopic cholecystectomy (LC) has been widely used by surgeons. However, the missed diagnosis of intraperitoneal malignant tumor may occur. If the malignancy exists, the changes of the abdominal environment or the laparoscopic operation might brought the cancer cells to abdominal cavity or wall, to more extreme condition, will be located in the navel, which is known as Sister Mary Joseph's nodule(SMJN). A 63-year-old female who had undergone cholecystectomy and choledocholithotomy ten months ago was hospitalized for upper abdominal pain. Laboratory examination indicated that most of tumor markers were increased. CT revealed a progressively enhanced mass around the left lobe bile duct, multiple enlarged lymph nodes in the abdominal cavity and nodular lesions were found under the costal margin of the right side of abdominal wall and the umbilicus. Biopsy of the nodules under the original surgical scar showed middle differentiated adenocarcinoma. In laparoscopic cholecystectomy, surgeons should not only focus on the local lesions, but also look around other the tissues and organs to avoid missing the abdominal malignant tumor. When atypical symptoms or abnormalities have been found pre-operation, all abdominal organs should be evaluated in detail to avoid missed diagnosis of potential malignant tumors. On the other hand, when there is a nodule in the umbilicus, all organs in abdomen should be examined to find the potential malignant tumor. Finally, multiple cholelithiasis in the left lobe of the liver should be regarded as a high risk factor for cholangiocarcinoma.

Benign Umbilical Tumors Resembling Sister Mary Joseph Nodule.

BACKGROUND: When physicians see an umbilical nodule, most of them instinctively recall the Sister Mary Joseph nodule. Therefore, dermatologists need to recognize umbilical dermatoses that can be mistaken for the Sister Mary Joseph nodules. This study aimed to describe the different kinds of benign umbilical tumors as well as elucidate the factors that can be used to distinguish the Sister Mary Joseph nodule from these tumors. METHODS: The "benign umbilical tumor" group included 19 patients, whereas the "Sister Mary Joseph nodule" group comprised 30 patients (2 from our department, 28 from PubMed search). We compared the clinical and dermoscopic findings between 2 groups. RESULTS: In the "benign umbilical tumor" group, the most common diagnosis was dermatofibroma (5/19), followed by keloid (3/19), and soft fibroma (3/19). These tumors had various colors (red, brown to black, and flesh colored) and exhibit characteristic surface changes (eg, verrucous changes in epidermal nevi and verrucae). Conversely, most Sister Mary Joseph nodules have an erythematous color, oozing or ulceration on the surface, and nearby satellite lesions. Furthermore, the dermoscopic findings of Sister Mary Joseph nodules showed a polymorphous vascular pattern and a white or milky-red, amorphous area. Benign lesions showed different dermoscopic patterns: pigment networks with white areas (dermatofibromas), thrombosed capillaries (verrucae), and the "pore sign" (epidermal cysts). CONCLUSIONS: Various cutaneous tumors can be mistaken for the Sister Mary Joseph nodule when they develop on the umbilicus; the clinical and dermoscopic differences found in this study may be useful for establishing a differential diagnosis.

Sister Mary Joseph's Nodule: Where Umbilicus Holds the Truth!

An umbilical metastasis from an internal visceral malignancy is defined as Sister Mary Joseph's nodule (SMJN), and, although a rare finding, it is a very poor prognostic indicator. We describe an interesting case of metastatic colon cancer with SMJN, emphasizing the significance of this classic finding. A 64-year-old female with a history of stage IV colon cancer with palliative right hemicolectomy and left hepatectomy presented to the hospital with nausea and abdominal discomfort. A computed tomography (CT) scan of the abdomen was performed, which showed small bowel obstruction secondary to metastatic tumor mass compressing the duodenum. She refused to undergo any chemotherapy or endoscopic intervention and was eventually discharged on hospice care. During the hospital stay an umbilical ulcerative lesion was also noted, which was violaceous, measuring 4.5 x 4 cm in size, firm in consistency with irregular borders, and tender to touch. Therefore, further history was obtained from the patient about it. Several months ago, she had developed localized swelling around the umbilicus, which gradually enlarged and ulcerated later. She eventually underwent the biopsy of that umbilical lesion, which confirmed it as SMJN with metastasis from the colonic primary. However, the patient refrained from the surgical intervention of the umbilical lesion. SMJN presents as a palpable periumbilical metastatic mass with diameter usually not exceeding 5 cm in size. It can be variable in color from violaceous to reddish brown. Once discovered, such lesions should be worked up with biopsy and imaging studies such as CT scan of the abdomen, as the nodule may be indicative of underlying malignancy or cancer recurrence. Its presence indicates a poor prognosis, with average survival time after diagnosis of SMJN of 10 months.

Sister Mary Joseph Nodule and Concomitant Pancreatitis as Initial Presentation of Pancreatic Adenocarcinoma - Case Report and Review of the Literature.

Umbilical metastasis [Sister Mary Joseph Nodule (SMJN)] is a rare presentation of visceral abdominal/pelvic malignancy. It is less commonly seen in metastatic pancreatic adenocarcinoma and there are only over a hundred cases to date in the literature on this topic. This article highlights a case of metastatic pancreatic adenocarcinoma presenting as SMJN and concomitant pancreatitis (which to the best of our knowledge is the first such report to date) with discussions regarding the etiopathogenesis of this phenomenon and presents a brief literature review on pancreatic adenocarcinoma presenting as SMJN.