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| Year : 2018 | Volume
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| Issue : 3 | Page : 457-458 |
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| An unusual intra-abdominal tumor of the gastrointestinal tract |
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Urmi Mukherjee, Anuj Khurana, Nayana Patil
Department of Laboratory Medicine, Max Super Speciality Hospital, Saket, New Delhi, India
Click here for correspondence address and email
| Date of Web Publication | 13-Jul-2018 |
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How to cite this article:
Mukherjee U, Khurana A, Patil N. An unusual intra-abdominal tumor of the gastrointestinal tract. Indian J Pathol Microbiol 2018;61:457-8 |
Editor,
Malignant neoplasms arising from the small bowel are a rare occurrence;[1],[2],[3],[4] with gastrointestinal stromal tumor (GIST) being the most common mesenchymal tumor described at that site. Clear-cell sarcoma synonymous with malignant melanoma of soft part is an uncommon soft-tissue neoplasm known for its aggressive behavior.[1],[2],[3],[4] We report an extremely uncommon case of a clear-cell sarcoma-like tumor of the gastrointestinal tract (CCSLGT).
A 35-year-old female presented elsewhere 3 years back with the complaints of diffuse abdominal pain, distention, on and off constipation for 10 months, for which she was managed on out patient department (OPD) basis with laxatives. There was a history of weight loss of 5 kg for 2 months. On radiological investigation, computed tomography (CT) scan abdomen revealed a small bowel tumor with features suggestive of recurrent subacute intestinal obstruction. She underwent a small bowel segment resection. Histopathological examination was suggestive of a jejunal sarcoma, further details of which were unavailable. The patient refused any adjuvant therapy. She was kept on regular follow-up with serial positron-emission tomography (PET) scans that did not reveal disease recurrence for 2 years. The latest PET scan revealed metabolically active nodular peritoneal deposit with maximum standard uptake value of 5.7. No other PET avid lesions ware discerned. The patient was explored laparoscopically with peritoneal deposit resection at our hospital. On gross examination, a nodular lesion measuring 3 cm in its largest dimension was identified and representative sections were taken.
On microscopic examination, it revealed an unencapsulated tumor comprising of monomorphic round cells arranged in lobular and nested pattern, separated by broad collagenous septae [Figure 1]a. The tumor cells showed open chromatin, inconspicuous nucleoli; moderate amount of pale eosinophilic to clear cytoplasm. Mitosis was found to be 3–5/10 hpf [Figure 1]b. No necrosis or evidence of angioinvasion was detected. | Figure 1: (a) Microphotograph showing monomorphic round cells arranged in lobular and nested pattern, separated by broad collagenous septae (H and E, ×100). (b) High-power view of cells with moderate amount of pale eosinophilic to clear cytoplasm, open chromatin, inconspicuous nucleoli, and mitosis (arrows) (H and E, ×400). (c) Immunohistochemistry showing S100 nuclear expression (DAB, ×400). (d) Microphotograph showing strong immunoreactivity to synaptophysin (DAB, ×400)
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Morphological diagnosis of a round cell neoplasm was followed by a panel of immunohistochemical markers. The tumor cells were positive for vimentin along with a diffuse nuclear expression S100 protein [Figure 1]c. They were immunonegative for pancytokeratin, CD45, desmin, SMA, CD117, CDK4, and MDM2. The tumor cells showed features of neuroendocrine differentiation with strong synaptophysin [Figure 1]d and traced CD56 expression; however, chromogranin was not expressed. Melanocytic markers (HMB45 and Melan A) were not expressed. The proliferative index was approximately 14%–15% in the most active areas. A final diagnosis of a CCSLGT was rendered.
CCSLGT was first described by Zabrano et al. as a distinct entity with only 38 cases reported so far. It is identified by a peculiar morphology like architectural cells are arranged in diffuse sheets without well-formed nests typical of CCS, the presence of CD68-positive multinucleated osteoclast-like giant cells distinct from tumor giant cells of CCS, and the absence of melanin pigment in all reported cases.[1],[3] CCSLGT shows expression of S100 protein with the absence of melanocyte-specific markers, such as HMB-45, Melan-A, tyrosinase, and microphthalmia transcription factor.[4] A focal expression of neural or neuroectodermal markers in IHC or electron microscopy suggests CCSLGT may be arising from neuroectodermal precursor cells that have lost the potential to differentiate along melanocytic lineage, hence proposed to be designated as “malignant gastrointestinal neuroectodermal tumor.”[5] Differential diagnosis includes adenocarcinoma, GIST, leiomyosarcoma, carcinoid tumor, and lymphoma which can be distinguished on the basis of an elaborate immunoprofile.[2] EWSR1-CREB1, and less frequently, EWSR1-ATF1 gene rearrangements are identified.[2],[4],[5] The same was not performed in the index case because of its unavailability in-house and in referral laboratories as this is a rare gene fusion test. We present this case to highlight its rarity and suggesting that this uncommon tumor to be kept as a differential diagnosis in tumors with unfamiliar histology and limited immunoexpression.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
 References | |  |
| 1. | Thway K, Judson I, Fisher C. Clear cell sarcoma-like tumor of the gastrointestinal tract, presenting as a second malignancy after childhood hepatoblastoma. Case Rep Med 2014;2014:984369.  |
| 2. | Wang J, Thway K. Clear cell sarcoma-like tumor of the gastrointestinal tract: An evolving entity. Arch Pathol Lab Med 2015;139:407-12. |
| 3. | Gahanbani Ardakani A, Boyle DJ, Elton C. Gastrointestinal clear cell sarcoma-like tumour of the ascending colon. Ann R Coll Surg Engl 2016;98:e37-9. |
| 4. | Yegen G, Güllüoǧlu M, Mete Ö, Önder S, Kapran Y. Clear cell sarcoma-like tumor of the gastrointestinal tract: A case report and review of the literature. Int J Surg Pathol 2015;23:61-7. |
| 5. | Raskin GA, Pozharisski KM, Iyevleva AG, Rikov IV, Orlova RV, Imyanitov EN, et al. Unusual clinical presentation of gastrointestinal clear cell sarcoma. Gastrointest Tumors 2015;2:83-8. |
Correspondence Address:
Nayana Patil
Department of Laboratory Medicine, Max Super Speciality Hospital, Saket, New Delhi - 110 017
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/IJPM.IJPM_658_17
[Figure 1] |
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| This article has been cited by | | 1 |
Malignant Gastrointestinal Neuroectodermal Tumour—Case Report with Review of Literature |
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