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OJHAS Vol. 10, Issue 1:
(Jan-Mar 2011) |
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Tubular Krukenberg Tumor with an Occult
Primary |
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Kavita Mardi, Associate Professor,
Vijay Kaushal, Professor & Head,
Navjot Kaur, Senir Resident,
Dept. of
Pathology, Indira Gandhi Medical
College, Shimla. |
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Address for Correspondence |
12-A, Type V Quarters,
GAD Colony,
Kasumpti,
Shimla, Himachal Pradesh, India.
E-mail: kavitamardi@yahoo.co.in |
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Mardi K, Kaushal V, Kaur N. Tubular Krukenberg Tumor with an Occult
Primary. Online J Health Allied Scs.
2011;10(1):18 |
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Submitted: Jan 9,
2011; Accepted: March 30, 2011; Published:
April 15, 2011 |
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| Abstract: |
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Tubular
Krukenberg tumor
with an occult primary can cause problems in histopathologic diagnosis, by
mimicking primary ovarian tumors. We present one such occurrence in a
32year old female who underwent surgery for bilateral malignant ovarian
tumor. Gross examination of both ovarian tumors revealed bosselated, smooth
outer surface with a few cysts on the surface. Cut surface was
grey-white to yellowish in colour with cysts filled with serous fluid
at the periphery. Microscopic examination revealed mucin laden signet
ring cells, predominantly showing tubular architecture within a
cellular ovarian stroma. The cytoplasm of these cells varied from
granular eosinophilic to pale vacuolated appearance and showed PAS and
mucicarmine positive mucin. Differential diagnosis with other primary
ovarian tumors is discussed.
Key Words:
Krukenberg tumors; Tubular
variant; Ovary
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Krukenberg tumors (KT)
are rare among ovarian metastases, but responsible for the most frequent
diagnostic confusions with primary ovarian cancers. Especially those
with an occult primary can cause diagnostic confusion with the primary
ovarian tumors. Distinction from the latter is of great importance as
misclassification of Krukenberg tumor as a primary ovarian tumor may
lead to suboptimal treatment of the patient. We report a case of tubular
Krukenberg tumor in a 32 years old female with an occult primary tumor
and discuss the diagnostic difficulties that arise with such an
occurrence.
A 32 year old female presented
with fullness of lower abdomen, polymenorrhagia and decreased appetite
since 2months. CT scan revealed a large well defined soft tissue density
mass lesion with heterogenous solid and cystic components in both uterine
adenexa measuring 16x11.5cm (right side) and 12x6cms (left side) respectively. In
addition there were gross ascitis and significant retroperitoneal lymphadenopathy. Possibility
of bilateral malignant sex cord stromal tumor was suggested. Serum levels
of CA-125 was also raised (1410u/ml). Patient underwent total abdominal
hysterectomy with bilateral salphingo-ophorectomy.
On gross examination, ovarian
tumors were measuring 17x11x9cms and 12x8x5cms respectively. Both
ovarian tumors showed bosselated, smooth outer surface with a few cysts
on the surface. Cut surface showed lobulated, grey white to yellowish
colour with cysts filled with serous fluid at the periphery.
Microscopic examination
of the sections from both ovaries showed mucin laden signet ring cells, predominantly
showing tubular architecture within a cellular ovarian stroma (Figure2). The
cytoplasm of these cells varied from granular eosinophilic to
pale vacuolated appearance and showed PAS and mucicarmine positive mucin. The
diagnosis of bilateral Tubular Krukenberg was given. Detailed radiographic
and endoscopic exploration of the digestive system of the patient did
not reveal any primary tumor.
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Figure 1:
Photograph of hysterectomy specimen with bilateral ovarian tumors
with bosselated outer surface |
Figure 2:
Photomicrograph revealing signet ring cells arranged in tubular pattern. (H&E,40x) |
Krukenberg-type tumors (KT) are rare among ovarian metastases. They are
responsible for the most frequent diagnostic confusions with primary
ovarian cancer. They are peculiar tumors with uncertain pathogenesis,
challenging etiological diagnosis and poorer prognosis for the primary.1 Krukenberg
tumor is a metastatic signet ring cell adenocarcinoma of the ovary,
accounting for 1% to 2% of all ovarian tumors.1 Stomach
is the primary site in most Krukenberg tumors (70%). Carcinomas of colon,
appendix, and breast (mainly invasive lobular carcinoma) are the next
most common primary sites. Rare cases of Krukenberg tumor originating
from carcinomas of the gallbladder, biliary tract, pancreas, small intestine,
ampulla of Vater, cervix, and urinary bladder/urachus have been reported.2
The interval between the diagnosis of a primary carcinoma and the subsequent
discovery of ovarian involvement is usually 6 months or less, but longer
periods have been reported. A history of a prior carcinoma of the stomach
or any other organ can be obtained in only 20% to 30% of the cases.3
In many cases, the primary tumor is very small and can escape detection.
In such a situation, diagnosis of Krukenberg tumor warrants careful
radiographic and endoscopic exploration of the digestive system in an
attempt to detect the primary carcinoma.In the present case,meticulous
radiographic and endoscopic investigation failed to detect any primary
carcinoma.
Novak and Woodruff4 have defined the criteria to qualify
a Krukenberg tumor as primary tumor of the ovary (Primary
krukenberg tumor). This includes
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Complete postmortem examination -
if
the patient was dead at the time of the case report, absence of primary
tumor in any organ except the ovary should be proved by a detailed autopsy
study.
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If the patient was living at the time of the case report,
and a surgical resection of the tumor was done, then the patient should
have survived for 5 years or longer. An analysis of 19 acceptable
cases reported in the literature favor the existence of primary
Krukenberg tumor of the ovary.5
While the entity of primary Krukenberg
tumor cannot be unequivocally denied, all women with typical Krukenberg
tumors should be considered as having metastatic carcinoma, usually from the
stomach, until proven otherwise.3 Our
patient is fine six months following surgery. However longer follow up
is needed to call it a primary Krukenberg tumor.
The diagnosis of Krukenberg tumors largely depends on the recognition
of its characteristic light microscopic features . However, Krukenberg
tumors may mimic other metastatic or primary ovarian tumors. Distinction
from the latter is of great importance as misclassification of Krukenberg
tumor as a primary ovarian tumor may lead to suboptimal treatment of
the patient. Tubular Krukenberg tumors (i.e., tumors in which the cells
are predominantly arranged in tubules) must be distinguished from other
ovarian tumors displaying annular or tubular pattern. The major tumor
in this group is Sertoli-Leydig cell tumor, particularly if intracellular
mucin is not evident on routine staining. Tubular Krukenberg tumors
are frequently bilateral, and compulsive sampling of the tumor usually
demonstrates the typical signet-ring cells filled with mucin.
The nuclei of tubular Krukenberg tumors are more atypical than those
within the tubules of Sertoli-Leydig cell tumors. Presence of signet
ring cells within the tubules is inconsistent with Sertoli-Leydig cell
tumor. In contrast to Krukenberg tumor, Sertoli-Leydig cell tumor stains
positive to inhibin but negative to cytokeratins or epithelial membrane
antigen.6 Other ovarian tumors with a tubular pattern that
can enter the differential diagnosis in this group include well-differentiated
endometrioid carcinoma (metastatic or primary), clear cell carcinoma
and tumors of wolffian origin. Differentiation can be made on the basis
of their characteristic histologic features.6 Bullon
A et al7 reviewed a series of 70 Krukenberg tumors
and found 13 cases with a predominant tubular pattern. Eleven of them
had been diagnosed by the referring pathologist as a tumor in the sex
cord-stromal category, usually a Sertoli-Leydig cell tumor and no diagnosis
was given in the other two cases. Three factors contributed to the erroneous
diagnoses in their cases which included: a prominent tubular pattern,
luteinization of the stroma of the tumor in five cases, and associated
virilization in two cases. Each tumor, however, contained typical signet-ring
cells that were readily demonstrable with mucicarmine stains. With
their experience ,they concluded that the diagnosis of Krukenberg tumor
must always be considered in the differential diagnosis of an ovarian
tumor with a tubular pattern even though endocrine manifestations are
present. Rarely, primary mucinous carcinomas of the ovary may contain
signet ring cells, but not in great number. However, careful consideration
of the clinical background, distribution of disease, gross characteristics
and spectrum of routine microscopic findings, will lead to the correct
diagnosis in the majority of cases and at the very least lead to formulation
of a considered differential diagnosis such that use of special techniques
may be judicious and those results placed in context of the time-honored
clinical and pathologic features.
Preoperative serum CA 125 levels in patients with Krukenberg tumors
can be elevated, though they subsequently decrease after tumor resection.8
On the basis of this observation, serum CA 125 level can be used for
(1) postoperative follow-up of patients for evaluation of complete resection
of the tumor, and (2) follow-up of patients with a history of primary
adenocarcinomas (gastrointestinal, in particular) for early detection
of ovarian metastasis. Serum CA 125 level also can help predict the
prognosis.
- Mates IN, Iosif C, Bănceanu G, Ionescu M, Peltecu G, Dinu D, Constantin A, Hoară P, Mitru C, Constantinoiu S. [Features of Krukenberg-type tumors--clinical study and review].
Chirurgia. 2008;103(1):23-38.
Hale RW. Krukenberg
tumor of the ovaries: a review of 81 records. Obstet Gynecol 1968;32:221-225.
Holtz F, Hart WR.
Krukenberg tumors of the ovary: a clinicopathological analysis of 27
cases. Cancer. 1982;50:2438-47.
Novak ER, Woodruff JD. Novak’s Gynaecologic and Obstetrical Pathology. 6th ed. Philadelphia. W.
B. Sauntlers Co. 1967. pp355-364.
Joshi V. Primary Krukenberg Tumor of ovary: Review of Literature and Case Report.
Cancer 1968;22:1199-1207.
Al-Agha OM, Nicastri AD. An
In-depth Look at Krukenberg Tumor: An Overview. Arch Pathol Lab Med. 2006;130:1725-1730.
Bullón
A Jr, Arseneau J, Prat J, Young RH, Scully RE. Tubular
Krukenberg tumor. A problem in histopathologic diagnosis.
Am J Surg Pathol 1981;5:225-232
Sugimoto Y, Endo K, Sakahara
H et al. Sequential measurement of serum CA 125 levels in Krukenberg's
tumor. Gan No Rinsho 1985;31:1893-1897.
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