Advanced colon adenocarcinoma with bone metastasis and krukenberg tumor
in a teen age: case report and review of literature
Abstract: colorectal cancer, one of the most common malignancies among adults, is rare in
adolescence. This low incidence coupled with non-specific
Introduction:
colorectal adenocarcinoma represents less than 1% of all neoplasms in the first two decades of
life [1]. Histologically, the aggressive mucinous carcinoma subtype accounts for most cases of
this cancer [1, 2–3]. Although colorectal carcinoma has a good prognosis in adults when
diagnosed early, in children, the rarity of the tumor and its high potential for dissemination
usually lead to late diagnosis and poor prognosis [4–5].
Colorectal cancer metastases occur predominantly in the liver and the lung , with extrahepatic
sites being far less common and equally distributed in brain, skin, and bone.
Krukenberg tumor (KT) is a metastatic signet ring cell adenocarcinoma of the ovary. It is
uncommon, accounting for 1% to 2% of all ovarian tumors. Stomach is the primary site in most
KT cases (70%). Carcinomas of colon, appendix, and breast (mainly invasive lobular carcinoma)
are the next most common primary sites.[6].
In this report, we describe a case of Krukenberg tumor in a 19-years-old girl with colon cancer
and simultaneously bone metastasis
Case report:
A 19-year-old girl presented with a 3-years history of increasing constipation, bleeding per
rectum and weight loss with no relevant past or family history. she was diagnosed as suffering
from simple constipation and hemorrhoids and discharged on laxatives. The symptoms
progressed and she was readmitted 2 years after with abdominal mass. Ultrasound tomography
was performed and diagnosis of simple ovarian mass was considered. She was referred to the
gynecologic consultation and underwent oraviectomy. The histopathological study revealed
mucinous adenocarcinoma of gastrointestinal origin. Few days after the ovariectomy, the patient
experienced intractable pain described as being like an electric shock and paraplegia. A diagnosis
of metastatic spinal cord compression was considered and computed tomography CT was
performed and showed huge metastatic mass destroying the right posterior body and pedicle, and
compressing the right posterior spinal cord, dorsal, and ventral nerve roots, dorsal root ganglion,
and spinal nerve with diffuse circumferential soft tissue thickening on the left colon region with
haziness of the surrounding fat. The patient underwent an emergency decompressive
laminectomy. The colonoscopy demontrated an ulcerative impassable stricture of the descending
colon. Pathologic examination of biopsies revealed moderate differentiated mucinous
adenocarcinoma. The patient’s condition continued to deteriorate in the post operative until she
died on 5th post operative day.
Discussion:
Although CRC is one of the most frequent tumors in adults, it rarely occurs before the age of 20
years,4–6 with an annual incidence of only one to two cases per one million people in the US,
accounting for only about 80 cases per year 7. CRC is more common in developed countries. It is
more frequent in Europe and North America than in Africa or Asia, with the exception of Japan.5
Well-defined CRC predisposition syndromes account for only about 3–5% of all cases of colon
cancer; they include Peutz-Jeghers syndrome, familial juvenile polyposis, hereditary mixed
polyposis syndrome, hereditary non-polyposis colon cancer, and familial adenomatous
polyposis.8 The vast majority of children reported with CRC were over 10 years of age;9
however, a few cases were a premature newborn with multiple congenital anomalies 10 and a 9-
month-old child reported with isolated CRC.10 CRC in children tends to present with similar
complaints as in adults, primarily with abdominal pain, but also hematochezia, altered bowel
habits, weight loss, and anemia. Children and adolescents also often present with acute
abdominal conditions, such as acute obstruction, perforation, or severe pain mimicking
appendicitis. In some pediatric reports, acute presentations, including intestinal obstruction and
acute pain mimicking appendicitis, account for almost 50% of presentations.9 Changes in bowel
habits, such as constipation or diarrhea, and in the caliber of stools may be observed before the
development of tarry stools, rectal bleeding, or other changes. There may be decreased appetite
and weight loss. The presentation of CRC is related to its primary site within the large bowel.
Tumors involving the cecum and descending colon may become bulky before symptoms appear.
Tumors of the rectum and sigmoid colon may be associated with changes in the caliber of the
stool, dyschezia, hematochezia, and anemia. The diagnosis of CRC in young patients is often
delayed because it is seldom suspected. Once the diagnosis is suspected, evaluation typically
includes abdominal X-rays, barium enema, CT, and eventually colonoscopy, which will show
either obstruction, narrowing of the colonic lumen, or an abdominal mass. Most colorectal
cancers in adults are moderately differentiated or well differentiated adenocarcinomas.
In
contrast, more than half of reported cases of childhood CRC are poorly differentiated mucinous
adenocarcinoma, and many are of the signet-ring cell type.
Metastases of colorectal carcinoma to bone is uncommon and usually occurs late in the disease
process usually after widespread metastatic disease . Data published by Kanthan et al.2 showed
eighty-three percent of patients with bone metastases also had either lung, liver or brain
metastases. This patient had multiple lung metastases in addition to the bony metastases
The most likely route for skeletal seeding is through Batson's plexus, a valveless system of veins
draining to the vertebral column, making it the most common site for skeletal metastasis. Other
frequent sites include the skull, pelvis, femur and humerus [4,9-11].
Krukenberg tumors are pathologically “signet ring cell” ovarian adenocarcinoma. They account
for 1-2% of all ovarian tumors world-wide. Women are typically diagnosed with Krukenberg
tumors in the perimenopausal fifth decade of life.10 Krukenberg tumor is more common in
premenopausal women than in postmenopausal women.11, 12 It is hypothesized that this young
age of diagnosis is related to the great vascularity of their ovaries, which facilitates vascular
metastasis. Kiyokawa T. and et al., analyzed 120 Krukenberg tumors. The patients’ average age
was 45 years with 43% of them under 40 years. Our patient was 19 years. Abdominal swelling or
pain usually accounted for the clinical presentation,13 in our case it was an autopalpable
abdominal mass. Krukenberg tumors are bilateral in 80% of cases. The route of metastasis from
the gastrointestinal tract to the ovaries is hypothesized to be via lymphatic. Krukenberg tumors
can be diagnosed before, after, or at the same time as diagnosis of the GI primary tumor. The
prognosis worsens when the primary tumor is identified after the metastasis to the ovary is
discovered.
In literature review we do not found any case of colon cancer with both Krukenberg tumor and
vertebral metastasis in the same time.
Table 1 shows the previously reported adenocarcinoma in children and adolescents
Conclusion:
As our knowledge we report first case of simultaneously two rare sites of colon cancer in a teenage patient. His cancer diagnosis was delayed by the physician’s failure to consider malignancy
in the differential diagnosis. Therefore, even in low-risk young patients, symptoms such as
unexplained abdominal pain, rectal bleeding, or changes in bowel habits should be considered
representative of significant colorectal lesions. This case warrants increased awareness and
aggressive pursuit of symptoms in young patients without any risk factors.
reports
Number of cases
Age (years)
sex
Site of tumors
pathology
Chana et al. (2001) India
1
12
male
rectum
Well-differentiated mucinous
adenocarcinoma
Jeong
et
al.(2008)
1
13
Ferrari et al.( 2008)
7
Median
female
rectosigmoid
Well-differentiated adenocarcinoma
2 males
Right colon
Mucinous adenocarcinoma (4 cases)
5 females
Splenic flexure (1 case)
Moderately differentiated adenocarcinoma (2 cases)
Korea
Italy
age
(12)
Left colon (1 case)
Poorly
differentiated
adenocarcinoma
with
neuroendocrine differentiation (1 case)
Sigmoid (3 cases)
Sharma et al.( 2009) India
2
14
Rectosigmoid
Mucinous adenocarcinoma
7 Males
Transverse colon (3 cases)
Mucinous adenocarcinoma (7 cases)
4 Females
Right colon (2 cases)
Adult form of non-mucinous adenocarcinoma (2
1 male
1 female
Salas-Valverde et al.(2009) Costa
11
Median age (11.6°)
Rica
cases)
Hepatic flexure (1 case)
Moderately differentiated adenocarcinoma (1
Splenic flexure (1 case)
case)
Left colon (1 case)
Sigmoid (1 case)
Poorly differentiated adenocarcinoma (1
case)
Multiple segments (2 cases)
Muccillo et al
(2010)
1
12
male
Right colon
Mucinous adenocarcinoma
Chattopadhyay et al. (2010) India
1
10
Female
Descending colon
Poorly differentiated mucinous adenocarcinoma
Malik and Kamath (2011) India
1
11
male
Rectum
Invasive
Mucinous adenocarcinoma
Brazil
poorly
differentiated
signet
ring
carcinoma grade 3
Agrawal et al.(2011) India
1
11
Male
Rectosigmoid
Ibrahim et al.(2011) Nigeria
2
16 and 18
2 Males
Unavailable
Unavailable
Our patient
1
19
Female
Descending colon
Moderate
differentiated
adenocarcinoma
mucinous