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OJHAS Vol. 11, Issue 1:
(Jan-Mar 2012) |
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Primary Signet-Ring Carcinoma
(Linitus Plastica) of the Colorectum presenting as Subacute Intestinal
Obstruction |
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Sunil V Jagtap, Dept. of Pathology,
Dhiraj B Nikumbh, Dept. of Pathology,
Ashok Y Kshirsagar, Dept.
of Surgery,
Swati S Jagtap, Dept.
of Physiology
Shamima, Dept. of Pathology,
KIMS University and KHMRC, Karad, Maharashtra, India. |
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Address for Correspondence |
Associate Professor,
Department of Pathology,
Krishna Institute of Medical Sciences,
University
of Karad - 415110,
Maharashtra, India.
E-mail:
drsvjagtap@gmail.com |
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Jagtap SV, Nikumbh DB, Khirsagar AY, Jagtap SS, Shamima. Primary Signet-Ring Carcinoma
(Linitus Plastica) of the Colorectum presenting as Subacute Intestinal
Obstruction. Online J Health Allied Scs.
2012;11(1):14 |
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Submitted: Feb 28,
2012;
Accepted: Mar 25, 2012; Published: Apr 15, 2012 |
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| Abstract: |
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Primary Signet-ring cell carcinoma (Linitus Plastica)
of the colon and rectum is a rare form of adenocarcinoma of the large
intestine and has been reported to have an extremely poor prognosis. We report a case of Primary Signet-ring cell carcinoma
of the colorectum in a thirty one year old man presented in Surgical
OPD of our hospital with chief complaints of persistent pain in abdomen
and vomiting since two days. Since the prognosis of primary signet ring cell carcinoma
(SRCC) is extremely poor (in view of more malignant behavior than ordinary
colorectal carcinoma), early diagnosis and aggressive treatment strategy
are necessary.
Key Words:
Signet ring cell carcinoma; Colorectal neoplasm;
Linitus plastica
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Signet ring cell carcinoma (SRCC) is uncommon in
the colon and rectum, with a reported incidence ranging from 0.1% to
0.8%.[1,2] It is a rare but distinctive malignancy of
the large bowel with more malignant behavior. More than 96% of the SRCC
arises in the stomach, with the remainder arising from other sites involving
colon, rectum, gall bladder, pancreas, urinary bladder and breast.[3] In general, SRCC shows the characteristic appearance of
“Linitus Plastica” and behaves more aggressively than other histological
types of carcinoma.[1,2] Primary SRCC were seen in younger patients (less than forty years
of age) as per Tung SY et al.[3] As symptoms usually appear late, SRCC are commonly detected at advanced
stages. Therefore, cases detected and treated at early stage are rare.
In this case report, we report a thirty one year old man with SRCC in
colorectum which was detected at early stage and treated aggressively.
A thirty one year old man presented in surgical outpatient
department of our hospital with chief complaints of persistent pain
in abdomen with vomiting since two days. He had past history of constipation
and altered bowel habits, off an don since 3 months. Patient was a chronic tobacco
chewer and potter by occupation with no history of diabetes
mellitus, tuberculosis, inflammatory bowel disease, hypertension or ischemic heart disease. Family history was
not relevant. Per abdominal examination revealed distension with vague
tenderness in epigastric and hypogastric region with free fluid and
sluggish peristalsis. Other systemic examination was within normal limit.
Per rectal examination did not reveal any abnormality. X ray chest was
within normal limit. Plain abdomen radiography suggestive of large bowel
obstruction and dilated bowel loops proximal to rectum. Gastroscopy
revealed no abnormality. Laboratory investigations revealed mild neutrophilic
leucocytosis with mildly increased creatinine. Rest of the biochemical
parameters were within normal limits. With above findings, clinical
diagnosis of subacute intestinal obstruction was made and laprotomy
was performed.
Laparotomy revealed distended sigmoid colon. Serosal
surface showed multiple tiny nodules of mucin. A stricture
was palpable in distal sigmoid colon. Radical surgical resection of
rectosigmoid colon with wide margin of 5 cm was done with end to end
anastomosis. Peritoneal seeding was noted. Stomach, liver and spleen
were normal. The resected rectosigmoid specimen was sent for histopathological
study.
Gross Features: Received a segment of recto-sigmoid measuring 15
cm in length, showing a constricted area measuring 2 cm in length and
6 cm away from distal surgical margin. Serosa showed congestion and
many mucinous nodules (Figure1).On cutting open, rectosigmoid showed
a large single encircling gray-white ulceroinfiltrative growth measuring
3x2 cm (Figure 2). Wall was thickened by tumor and growth was seen reaching
upto serosa. Cut section of the tumor mass was gray-white with mucinous
areas. Also received peritoneal whitish nodules measuring 0.3 to 0.6
cm. On cutting open revealed gray-white, mucinous areas.
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Figure 1: Gross specimen of
resected rectosigmoid
with constricted area and serosal tiny mucinous nodules.
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Figure 2: Cut surface of the
rectosigmoid showing
encircling gray white ulceroinfiltrative mass. |
Light Microscopy: Multiple sections through rectosigmoid showed
large intestinal mucosa with extensive areas of ulceration and tumor
arising from it and infiltrating deeper tissue (Figure 3). The tumor
was composed of neoplastic cells arranged in thick cords, nests, lobules
and ill formed glands. Individual tumor cells are large, round to oval
having mild pleomorphic hyperchromatic nuclei with abundant vacuolated
or eosinophilic cytoplasm pushing the nuclei to periphery (signet ring
cells) (Figure 4, 5). Many tumor cells floating in the pools of extracellular
mucin were noted. Solid areas of tumor at places were also seen. Tumor
was seen infiltrating full thickness of mucosa and over serosal surfaces.
Occasional tumor emboli, perineural infiltration were seen. Sections
from peritoneal nodules showed fibroadipose tissue with SRCC. Both the
surgical margins were free from tumor. The final histopathological diagnosis
of Primary Signet ring cell carcinoma was given. The patient was on
regular follow up.
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Fig 3: Photomicrograph showing large intestinal mucosa with
ulceration and tumor arising from it and infiltrating the deeper
structures. (H&E x100). |
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Figures 4,5:
Photomicrographs showing tumor cells arranged in thick cords,
nests and ill defined glands with characteristic signet ring cell
tumor cells. (H&Ex400). |
Primary Signet ring cell carcinoma (SRCC) of the
colon and rectum is a rare variant of colorectal adenocarcinoma. SRCC
has an aggressive clinical course and a poor prognosis.[4] There is high incidence of peritoneal metastases and relatively low
incidence of hepatic metastases, a characteristic feature distinguishing
colorectal signet ring cell carcinoma from non-signet colorectal carcinoma.[4]
SRCC has been described in virtually all mucinous
organs of the body, most frequently in stomach, but also in urinary
bladder, prostate, breast, lung, and pancreas etc.[5] Within the gastrointestinal tract, up to
96% of SRCC are found in stomach.[6] In the colon, although small foci of signet ring cells can
be seen in conventional adenocarcinoma, a primary SRCC with greater
than 50% of tumor cells being signet rings, is extremely uncommon.[5-7]
Primary signet ring cell carcinoma of the colon and
rectum as first described by Laufmann and Sufir in 1951 is rare entity
[8]. The reported incidence is 0.1 to 0.8% only.[1,2] The histological appearance of the tumor is characterized by cells
with abundant intracytoplasmic mucin and peripherally placed nuclei.
SRCC of the colon and rectum are usually diagnosed at an advanced stage,
because symptoms usually develop late. Thereafter cancers limited to
the mucosal and submucosal layers are rarely detected, as seen in our
case.
Primary SRCC of colorectum is diagnosed when the
following criteria are satisfied:
- The tumor is primary.
- Histological material is adequate.
- Signet ring cells present more than 50% of the cancer.[7]
All the three criteria were satisfied in our case.
In our case, clinical history, gastro copy and laparotomy ruled out a
primary growth in the stomach. Macroscopically SRCC shows annularly
thickened and rigid bowel wall in long segment and stenotic bowel lumen,
providing a linitus plastica appearance. Microscopically poorly differentiated
or signet ring cells are seen. Peritoneal dissemination is very common.[9] Our patient had all these features.
Immunostaining profiles for CK 7 and CK 20 have been
used to characterize and differentiate SRCC of breast, stomach and colon.[10,11] CK 20 is a low molecular weight cytokeratin that is
normally expressed in the gastrointestinal epithelium, urothelium and
in Merkel cells.[12] Ck 7 is expressed by tumors of the
lungs, ovary, endometrium and breast but not of the lower gastrointestinal
tract.[4]
Primary signet ring cell carcinoma of colorectum
is a rare entity with extremely poor prognosis in view of more malignant
behaviour than other colorectal carcinoma. Early diagnosis and
aggressive treatment strategy are necessary for better management of patients.
- Giacchero A, Aste H, Barachhini P, Conio M, Fulcheri
E, Laperto G, Tanzi R. Primary signet-ring cell carcinoma of large bowel.
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Almagro UA. Primary signet-ring cell carcinoma of
the colon. Cancer. 1983;52:1453-1457.
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Tung SY, Wu CS, Chen PC. Primary signet-ring cell
carcinoma of colorectum :an age and sex matched controlled study. Am J Gastroenterol.
1996;91:2195-2199.
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Sim HL, Tan KY, Poon PL, Cheng A. Primary rectal
signet-ring cell carcinoma with peritoneal dissemination and gastric
secondaries. World J Gastroenterol. 2008;14(3):2118-2120.
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Maeharn Y, Sakaguchi Y, Moriguchi S et al. Signet
ring carcinoma of the stomach. Cancer. 1992;69:1645-1650.
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Tendon M, Sostek M, Klein MA. Focus of signet ring-cell
carcinoma in an adenoma of the sigmoid colon. Arch Pathol Lab Med. 1999;123:957-959.
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Messerini L, Palomba A, Zampi G. Primary signet-ring
cell carcinoma of the colon and rectum. Dis Colon Rectum. 1995;38:1189-1192.
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Laufman H, Safir O. Primary linitus plastica type
of carcinoma of the colon. AMA Arch Surg. 1951;62:79-91.
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Nakahara H, Ishikawa T, Itubashi M, Hirota T. Diffusely
infiltrating colorectal carcinoma of linitus plastic and lymphangiosis
type. Cancer. 1992;69:901-906.
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Goldstein NS, Long A, Kuan SF, Hart J. Colon signet
ring-cell adenocarcinoma: immunohistochemical characterization and comparison
with gastric and typical colon adenocarcinomas. Appl Immunohistochem
Mol Morphol. 2008;8:183-188.
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Tot T. The role of cytokeratins 20 and 7 and estrogen
receptor analysis in separation of metastatic lobular carcinoma of the
breast and metastatic signet-ring cell carcinoma of the gastrointestinal
tract. APMIS. 2000;108:467-472.
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Varadhachary GR, Abbnizzese JL, Lenzi R. Diagnostic
strategies for unknown primary cancer. Cancer. 2004;100:1776-1785.
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