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CASE SERIES
Sister Mary Joseph Nodule A Tip of an Iceberg Marek Wroński, MD, PhD, Andrzej Kluciński, MD, Ireneusz W. Krasnodębski, MD, PhD
A Sister Mary Joseph nodule represents a cutaneous metastasis into the umbilicus. This clinical sign of intra-abdominal malignancy is frequently overlooked or misinterpreted by both patients and their physicians. We report 4 patients with a Sister Mary Joseph nodule. The umbilical metastases appeared sonographically as hypoechoic masses with irregular margins and small internal hyperechoic foci. Further evaluation revealed disseminated malignancy, and the umbilical nodule was just “a tip of an iceberg.” Key Words—gastrointestinal neoplasm; gastrointestinal ultrasound; Sister Mary Joseph nodule; sonography; umbilical metastasis
A
Sister Mary Joseph nodule is a clinical sign that is frequently overlooked or misinterpreted on physical examination. This lesion is a rare cutaneous metastasis from internal malignancy. This sign is a late manifestation of a malignant process and represents an advanced stage of the disease. The clinical recognition of umbilical metastases may be difficult, and sonographic assessment can prove helpful in confirming the diagnosis. However, the sonographic appearance of Sister Mary Joseph nodules has rarely been described in the literature. We report 4 patients with a Sister Mary Joseph nodule who were evaluated sonographically. The sonographic features of the umbilical metastases are discussed in relation to other more common lesions that involve the umbilicus.
Case Series Received March 18, 2013, from the Department of General, Gastroenterological, and Oncological Surgery, Medical University of Warsaw, Warsaw, Poland. Revision requested April 8, 2013. Revised manuscript accepted for publication July 17, 2013. Address correspondence to Marek Wroński, MD, PhD, Department of General, Gastroenterological, and Oncological Surgery, Medical University of Warsaw, Ulica Banacha 1A, 02- 097 Warsaw, Poland. E-mail: [email protected] doi:10.7863/ultra.33.3.531
Sonographic evaluations were performed with a Famio 8 scanner (Toshiba Medical Systems Co, Ltd, Tokyo, Japan) and an 8–12MHz linear array transducer. The clinical and sonographic findings in 4 patients with Sister Mary Joseph nodule are summarized in Table 1. All of our patients presented with gastrointestinal symptoms, and a nodule was palpated within the umbilicus on physical examination. In 1 patient, the skin of the umbilicus was normal. In another patient, the umbilicus was apparently normal except for a whitish central nodule. In 2 patients, the skin was indurated and erythematous with a granular surface (Figures 1A and 2A). In 1 patient, a painless umbilical tumor had been present for 3 months before the
©2014 by the American Institute of Ultrasound in Medicine | J Ultrasound Med 2014; 33:531–534 | 0278-4297 | www.aium.org
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Wroński et al—Sister Mary Joseph Nodule
diagnosis of pancreatic cancer was finally established. In 3 patients, the umbilical nodules remained unnoticed by the patients themselves and their primary care physicians until medical consultation in our institution. In this series, the umbilical metastases appeared sonographically as well-defined hypoechoic masses with irregular margins (Figures 1B and 2B). The internal echo structure of the nodules was heterogeneous with tiny hyperechoic foci. The lesions extended from the dermis down to the peritoneum. The rectus abdominis muscle was not infiltrated in any of the cases, nor did the nodule involve the bowels located beneath the umbilicus. The lesions had lobulated or spiculated projections infiltrating the subcutaneous fat, but the fatty tissue adjacent to the nodule remained grossly unaffected. The umbilical metastases appeared on computed tomography as well-defined hyperdense tumors (Figure 2C). Interestingly, none of the Sister Mary Joseph nodules in this series had been recognized on computed tomography, although a post hoc review of the scans confirmed its presence in each case. Pathologic examination revealed uniform adenocarcinoma, and all the patients presented at the stage of dissemination. The primary tumor involved the pancreas in 3 cases, and the primary focus was unknown in 1 case (probably biliary or pancreatic).
Discussion This umbilical metastasis was named after Sister Mary Joseph, who was first to suggest an association between a periumbilical nodule and internal malignancy. She was a superintendent nurse at the Mayo Clinic. The eponym was introduced by British surgeon Hamilton Bailey in 1949.1 Umbilical metastases may result from hematogenous, lymphatic, or local spread. The umbilicus has some anatomic peculiarities reflecting its embryologic development, which might predispose to metastases. Ching and Lai2 suggested that connection of the umbilicus with various embryologic remnants and its abundant vascular and lymphatic supply facilitate migration of tumor cells to the umbilicus. On the other hand, because of the incomplete fascial structure of the umbilicus and lack of a muscular layer, the transversalis fascia represents the only barrier to direct spread of peritoneal metastases in this part of the abdominal wall. The fact that all of the patients in our series had simultaneous multiple metastatic deposits seeding the peritoneum favors local infiltration as the route of dissemination for Sister Mary Joseph nodules. However, the exact pathogenesis of these metastases is still not known. A Sister Mary Joseph nodule most often originates from gastric adenocarcinoma in men and from ovarian cancer in women.3 The high rate of pan-
Table 1. Clinical and Sonographic Characteristics of the Patients With a Sister Mary Joseph Nodule
Case
Sex/ Age, y
Gross Appearance of Umbilicus
Sonographic Features of Umbilical Nodule
Additional Sonographic Findings
Nodule Size, mm
Type of Malignancy
Primary Tumor
1
M/64
Elevated, erythematous with granular surface
Hypoechoic elongated mass with spiculated margins, small internal hyperechoic foci
Ascites, metastatic inguinal lymph nodes
18
Adenocarcinoma
Unknown (possible pancreatic or biliary origin)
2
F/56
Indurated with erythematous central part
Hypoechoic oval mass with irregular margins, small internal hyperechoic foci
Pancreatic tumor infiltrating stomach
16
Adenocarcinoma
Body of pancreas
3
M/59
5-mm whitish central nodule
Hypoechoic irregular mass with small internal hyperechoic foci
Pancreatic tumor, ascites
20
Adenocarcinoma
Tail of pancreas
4
M 73
Normal
Hypoechoic beanlike mass with poorlydefined margins, small internal hyperechoic foci
Pancreatic tumor, ascites, intraperitoneal deposits
19
Adenocarcinoma
Body of pancreas
F indicates female; and M, male.
532
J Ultrasound Med 2014; 33:531–534
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Wroński et al—Sister Mary Joseph Nodule
Figure 1. Umbilical metastasis in a 64-year-old man with intraperitoneal dissemination from an unknown primary focus. A, Gross appearance of the umbilicus showing a granular and erythematous surface. B, Grayscale sonogram showing a hypoechoic elongated mass with spiculated margins and small internal hyperechoic foci (arrow). Of note, the subcutaneous tissue adjacent to the nodules does not show features of inflammation. F indicates subcutaneous fat; and M, rectus abdominis muscle. A
B
A
Figure 2. Umbilical metastasis in a 56-year-old woman with pancreatic adenocarcinoma. A, Gross appearance of the umbilicus showing an indurated and erythematous central part. B, Grayscale sonogram showing a hypoechoic oval mass with irregular margins and small internal hyperechoic foci (arrow). No inflammation of the tissues surrounding the nodule is shown. C, Computed tomogram showing a hyperdense tumor in the umbilicus (arrow). Abbreviations are as in Figure 1.
B
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C
533
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Wroński et al—Sister Mary Joseph Nodule
creatic cancer in our series reflects a referral bias because our institution is a tertiary center for pancreatic diseases. A palpable nodule in the umbilicus may be present for several months before the diagnosis of a neoplasm is finally established, as was the case in one of our patients. More commonly, the nodule remains unnoticed by patients or neglected by their physicians. Computed tomography usually shows a hyperdense contrast-enhancing tumor in the umbilicus.2 However, it is often overlooked by radiologists, as occurred in all of our patients, which was probably due to the low index of suspicion. The sonographic appearance of Sister Mary Joseph nodules has rarely been reported in the literature.2,3 The gross view of a Sister Mary Joseph nodule on physical examination is often misleading because the skin overlying the lesion can be normal or shows erythema. Cutaneous erythema gives a false impression of inflammation or even suggests an abscess of the umbilicus. However, Sister Mary Joseph nodules are painless, unlike inflammatory lesions. On sonography, periumbilical cellulitis appears as hyperechoic fat within the affected subcutaneous tissue, sometimes with a “cobblestone” appearance,4 but the fascial and muscular layers usually remain unchanged. Sonographically, an umbilical abscess is a hypoechoic and grossly heterogeneous lesion with variable fluid contents. Moreover, the adjacent fat also shows diffuse hyperechogenicity typical of cellulitis. In contrast, the fatty tissue surrounding the Sister Mary Joseph nodules in this series remained unaffected, without any sonographic signs of inflammation. The recognition of a Sister Mary Joseph nodule warrants further imaging evaluation because disseminated malignancy is usually found within the abdominal cavity. Thorough transabdominal sonography usually shows the primary tumor, with ascites or intraperitoneal deposits corresponding to a diffuse malignant infiltration; thus, a Sister Mary Joseph nodule represents just “a tip of an iceberg.” In conclusion, a solid hypoechoic mass in the umbilicus without any sonographic features of inflammation involving the adjacent soft fatty tissue might possibly suggest the diagnosis of a Sister Mary Joseph nodule, and the primary tumor and other metastases should be sought.
3.
4.
Panaro F, Andorno E, Di Domenico S, et al. Sister Joseph’s nodule in a liver transplant recipient: case report and mini-review of literature. World J Surg Oncol 2005; 3:4. Chau CL, Griffith JF. Musculoskeletal infections: ultrasound appearances. Clin Radiol 2005; 60:149–159.
References 1.
2.
534
Shen Z, Yang X, Chen L, Hao F, Zhong B. Sister Mary Joseph’s nodule as a diagnostic clue to metastatic colon carcinoma. J Clin Oncol2009; 27:e1– e2. Ching AS, Lai CW. Sonography of umbilical metastasis (Sister Mary Joseph’s nodule): from embryology to imaging. Abdom Imaging 2002; 27:746–749.
J Ultrasound Med 2014; 33:531–534
CASE SERIES
Sister Mary Joseph Nodule A Tip of an Iceberg Marek Wroński, MD, PhD, Andrzej Kluciński, MD, Ireneusz W. Krasnodębski, MD, PhD
A Sister Mary Joseph nodule represents a cutaneous metastasis into the umbilicus. This clinical sign of intra-abdominal malignancy is frequently overlooked or misinterpreted by both patients and their physicians. We report 4 patients with a Sister Mary Joseph nodule. The umbilical metastases appeared sonographically as hypoechoic masses with irregular margins and small internal hyperechoic foci. Further evaluation revealed disseminated malignancy, and the umbilical nodule was just “a tip of an iceberg.” Key Words—gastrointestinal neoplasm; gastrointestinal ultrasound; Sister Mary Joseph nodule; sonography; umbilical metastasis
A
Sister Mary Joseph nodule is a clinical sign that is frequently overlooked or misinterpreted on physical examination. This lesion is a rare cutaneous metastasis from internal malignancy. This sign is a late manifestation of a malignant process and represents an advanced stage of the disease. The clinical recognition of umbilical metastases may be difficult, and sonographic assessment can prove helpful in confirming the diagnosis. However, the sonographic appearance of Sister Mary Joseph nodules has rarely been described in the literature. We report 4 patients with a Sister Mary Joseph nodule who were evaluated sonographically. The sonographic features of the umbilical metastases are discussed in relation to other more common lesions that involve the umbilicus.
Case Series Received March 18, 2013, from the Department of General, Gastroenterological, and Oncological Surgery, Medical University of Warsaw, Warsaw, Poland. Revision requested April 8, 2013. Revised manuscript accepted for publication July 17, 2013. Address correspondence to Marek Wroński, MD, PhD, Department of General, Gastroenterological, and Oncological Surgery, Medical University of Warsaw, Ulica Banacha 1A, 02- 097 Warsaw, Poland. E-mail: [email protected] doi:10.7863/ultra.33.3.531
Sonographic evaluations were performed with a Famio 8 scanner (Toshiba Medical Systems Co, Ltd, Tokyo, Japan) and an 8–12MHz linear array transducer. The clinical and sonographic findings in 4 patients with Sister Mary Joseph nodule are summarized in Table 1. All of our patients presented with gastrointestinal symptoms, and a nodule was palpated within the umbilicus on physical examination. In 1 patient, the skin of the umbilicus was normal. In another patient, the umbilicus was apparently normal except for a whitish central nodule. In 2 patients, the skin was indurated and erythematous with a granular surface (Figures 1A and 2A). In 1 patient, a painless umbilical tumor had been present for 3 months before the
©2014 by the American Institute of Ultrasound in Medicine | J Ultrasound Med 2014; 33:531–534 | 0278-4297 | www.aium.org
3303jum531-552onine_Layout 1 2/19/14 1:39 PM Page 532
Wroński et al—Sister Mary Joseph Nodule
diagnosis of pancreatic cancer was finally established. In 3 patients, the umbilical nodules remained unnoticed by the patients themselves and their primary care physicians until medical consultation in our institution. In this series, the umbilical metastases appeared sonographically as well-defined hypoechoic masses with irregular margins (Figures 1B and 2B). The internal echo structure of the nodules was heterogeneous with tiny hyperechoic foci. The lesions extended from the dermis down to the peritoneum. The rectus abdominis muscle was not infiltrated in any of the cases, nor did the nodule involve the bowels located beneath the umbilicus. The lesions had lobulated or spiculated projections infiltrating the subcutaneous fat, but the fatty tissue adjacent to the nodule remained grossly unaffected. The umbilical metastases appeared on computed tomography as well-defined hyperdense tumors (Figure 2C). Interestingly, none of the Sister Mary Joseph nodules in this series had been recognized on computed tomography, although a post hoc review of the scans confirmed its presence in each case. Pathologic examination revealed uniform adenocarcinoma, and all the patients presented at the stage of dissemination. The primary tumor involved the pancreas in 3 cases, and the primary focus was unknown in 1 case (probably biliary or pancreatic).
Discussion This umbilical metastasis was named after Sister Mary Joseph, who was first to suggest an association between a periumbilical nodule and internal malignancy. She was a superintendent nurse at the Mayo Clinic. The eponym was introduced by British surgeon Hamilton Bailey in 1949.1 Umbilical metastases may result from hematogenous, lymphatic, or local spread. The umbilicus has some anatomic peculiarities reflecting its embryologic development, which might predispose to metastases. Ching and Lai2 suggested that connection of the umbilicus with various embryologic remnants and its abundant vascular and lymphatic supply facilitate migration of tumor cells to the umbilicus. On the other hand, because of the incomplete fascial structure of the umbilicus and lack of a muscular layer, the transversalis fascia represents the only barrier to direct spread of peritoneal metastases in this part of the abdominal wall. The fact that all of the patients in our series had simultaneous multiple metastatic deposits seeding the peritoneum favors local infiltration as the route of dissemination for Sister Mary Joseph nodules. However, the exact pathogenesis of these metastases is still not known. A Sister Mary Joseph nodule most often originates from gastric adenocarcinoma in men and from ovarian cancer in women.3 The high rate of pan-
Table 1. Clinical and Sonographic Characteristics of the Patients With a Sister Mary Joseph Nodule
Case
Sex/ Age, y
Gross Appearance of Umbilicus
Sonographic Features of Umbilical Nodule
Additional Sonographic Findings
Nodule Size, mm
Type of Malignancy
Primary Tumor
1
M/64
Elevated, erythematous with granular surface
Hypoechoic elongated mass with spiculated margins, small internal hyperechoic foci
Ascites, metastatic inguinal lymph nodes
18
Adenocarcinoma
Unknown (possible pancreatic or biliary origin)
2
F/56
Indurated with erythematous central part
Hypoechoic oval mass with irregular margins, small internal hyperechoic foci
Pancreatic tumor infiltrating stomach
16
Adenocarcinoma
Body of pancreas
3
M/59
5-mm whitish central nodule
Hypoechoic irregular mass with small internal hyperechoic foci
Pancreatic tumor, ascites
20
Adenocarcinoma
Tail of pancreas
4
M 73
Normal
Hypoechoic beanlike mass with poorlydefined margins, small internal hyperechoic foci
Pancreatic tumor, ascites, intraperitoneal deposits
19
Adenocarcinoma
Body of pancreas
F indicates female; and M, male.
532
J Ultrasound Med 2014; 33:531–534
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Wroński et al—Sister Mary Joseph Nodule
Figure 1. Umbilical metastasis in a 64-year-old man with intraperitoneal dissemination from an unknown primary focus. A, Gross appearance of the umbilicus showing a granular and erythematous surface. B, Grayscale sonogram showing a hypoechoic elongated mass with spiculated margins and small internal hyperechoic foci (arrow). Of note, the subcutaneous tissue adjacent to the nodules does not show features of inflammation. F indicates subcutaneous fat; and M, rectus abdominis muscle. A
B
A
Figure 2. Umbilical metastasis in a 56-year-old woman with pancreatic adenocarcinoma. A, Gross appearance of the umbilicus showing an indurated and erythematous central part. B, Grayscale sonogram showing a hypoechoic oval mass with irregular margins and small internal hyperechoic foci (arrow). No inflammation of the tissues surrounding the nodule is shown. C, Computed tomogram showing a hyperdense tumor in the umbilicus (arrow). Abbreviations are as in Figure 1.
B
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C
533
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Wroński et al—Sister Mary Joseph Nodule
creatic cancer in our series reflects a referral bias because our institution is a tertiary center for pancreatic diseases. A palpable nodule in the umbilicus may be present for several months before the diagnosis of a neoplasm is finally established, as was the case in one of our patients. More commonly, the nodule remains unnoticed by patients or neglected by their physicians. Computed tomography usually shows a hyperdense contrast-enhancing tumor in the umbilicus.2 However, it is often overlooked by radiologists, as occurred in all of our patients, which was probably due to the low index of suspicion. The sonographic appearance of Sister Mary Joseph nodules has rarely been reported in the literature.2,3 The gross view of a Sister Mary Joseph nodule on physical examination is often misleading because the skin overlying the lesion can be normal or shows erythema. Cutaneous erythema gives a false impression of inflammation or even suggests an abscess of the umbilicus. However, Sister Mary Joseph nodules are painless, unlike inflammatory lesions. On sonography, periumbilical cellulitis appears as hyperechoic fat within the affected subcutaneous tissue, sometimes with a “cobblestone” appearance,4 but the fascial and muscular layers usually remain unchanged. Sonographically, an umbilical abscess is a hypoechoic and grossly heterogeneous lesion with variable fluid contents. Moreover, the adjacent fat also shows diffuse hyperechogenicity typical of cellulitis. In contrast, the fatty tissue surrounding the Sister Mary Joseph nodules in this series remained unaffected, without any sonographic signs of inflammation. The recognition of a Sister Mary Joseph nodule warrants further imaging evaluation because disseminated malignancy is usually found within the abdominal cavity. Thorough transabdominal sonography usually shows the primary tumor, with ascites or intraperitoneal deposits corresponding to a diffuse malignant infiltration; thus, a Sister Mary Joseph nodule represents just “a tip of an iceberg.” In conclusion, a solid hypoechoic mass in the umbilicus without any sonographic features of inflammation involving the adjacent soft fatty tissue might possibly suggest the diagnosis of a Sister Mary Joseph nodule, and the primary tumor and other metastases should be sought.
3.
4.
Panaro F, Andorno E, Di Domenico S, et al. Sister Joseph’s nodule in a liver transplant recipient: case report and mini-review of literature. World J Surg Oncol 2005; 3:4. Chau CL, Griffith JF. Musculoskeletal infections: ultrasound appearances. Clin Radiol 2005; 60:149–159.
References 1.
2.
534
Shen Z, Yang X, Chen L, Hao F, Zhong B. Sister Mary Joseph’s nodule as a diagnostic clue to metastatic colon carcinoma. J Clin Oncol2009; 27:e1– e2. Ching AS, Lai CW. Sonography of umbilical metastasis (Sister Mary Joseph’s nodule): from embryology to imaging. Abdom Imaging 2002; 27:746–749.
J Ultrasound Med 2014; 33:531–534
