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Year : 2022  |  Volume : 18  |  Issue : 4  |  Page : 885-897

Malignant gastrointestinal neuroectodermal tumor: A case-based review of literature

1 Department of Pathology and Lab Medicine, AIIMS, Bhubaneswar, Odisha, India
2 Department of Surgical Oncology, AIIMS, Bhubaneswar, Odisha, India

Date of Submission05-Oct-2019
Date of Decision29-Aug-2020
Date of Acceptance10-Sep-2020
Date of Web Publication17-Jul-2021

Correspondence Address:
Amit Kumar Adhya
Department of Pathology and Lab Medicine, AIIMS, Bhubaneswar, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcrt.JCRT_829_19

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 > Abstract 

Malignant gastrointestinal (GI) neuroectodermal tumor is an extremely rare entity that was first described by Zambrano et al. in 2003 as “clear cell sarcoma (CCS)-like tumor of the GI tract.” It shares some of the histopathological features of CCS but lacks the immunohistochemical (IHC) reactivity for melanocytic markers. Most mesenchymal neoplasms of the GI tract belong to the category of GI stromal tumors and are characterized by the IHC expression of c-KIT. In cases, without detectable KIT receptor expression, several differential diagnoses have to be taken into consideration. In this article, we describe such a case and present a review of all the reported cases till date. We also present the current available knowledge on its pathology and molecular genetics along with the limitations in its diagnosis. Here, we report a case of a 32-year-old man with a tumor of the small bowel composed of polygonal tumor cells arranged in solid nests, alveolar pattern, and pseudopapillary and admixed with numerous osteoclast-like multinucleated giant cells. Immunohistochemically, the tumor cells strongly expressed S-100 protein only. HMB-45, melan-A, CD117, cytokeratin, desmin, smooth muscle actin, and CD-34 were absent. Ki-67 index was 15%. The diagnosis was further confirmed by fluorescence in situ hybridization (FISH) demonstrating the presence of EWSR1 (22q12) translocation. A final diagnosis of malignant gastroneuroectodermal tumor was rendered. The patient is disease-free for 20 months of postsurgery. The diagnosis of this entity should be considered in the presence of S-100-positivity and multinucleated osteoclastic giant cells and the absence of melanocytic differentiation in a tumor arising from GI tract. Further confirmation can be done by performing FISH analysis.

Keywords: Clear cell sarcoma, fluorescence in situ hybridization, multinucleated giant cells, translocation

How to cite this article:
Mishra P, Biswas D, Pattnaik SA, Patra S, Muduly DK, Balasubiramaniyan V, Adhya AK. Malignant gastrointestinal neuroectodermal tumor: A case-based review of literature. J Can Res Ther 2022;18:885-97

How to cite this URL:
Mishra P, Biswas D, Pattnaik SA, Patra S, Muduly DK, Balasubiramaniyan V, Adhya AK. Malignant gastrointestinal neuroectodermal tumor: A case-based review of literature. J Can Res Ther [serial online] 2022 [cited 2022 Oct 5];18:885-97. Available from: https://www.cancerjournal.net/text.asp?2022/18/4/885/321714

 > Introduction Top

Clear cell sarcoma (CCS) has been conventionally known to be a tumor of adolescents involving deep soft tissues, tendons, aponeuroses, and fascia of lower extremities, first described by Enzinger as CCS of tendons and aponeuroses (CCSTA).[1] Ekfors et al. observed a duodenal tumor displaying similar features as seen in CCSTA and reported it as CCS of the gastrointestinal tract (CCS-GI).[2] Bridge et al. noted a distinct chromosomal translocation t (12;22)(q13, q12) involving EWSR1-activating transcription factor 1 (ATF1) in CCSTA, which is also shared by the tumors of CCS-GI.[3] This makes them an entity different from malignant melanoma. Subsequently, few cases primarily arising from the GI tract with clear cell morphology and immunophenotyping and sharing the same molecular features with CCSTA were reported.[2] Many of these tumors lacked evidence of melanocytic differentiation both immunohistochemically and ultrastructurally and hence was reported as CCS-like tumor of the GI tract.[4] In fact, in 2012, Stockman et al. reviewed 16 cases of clear cell sarcoma like tumor of GI tract (CCSLTGT) and observed that though these tumors resemble CCSTA, they were different in many respects and hence proposed the term as “malignant gastrointestinal neuroectodermal tumor” (GNET).[5] In view of their rarity and their close resemblance to other common tumors, GNET may pose both diagnostic and therapeutic challenges. In this article, we describe a case of GNET in a middle-aged male giving special emphasis on the diagnostic challenges and molecular basis and also present a review of world literature of all such cases reported till date.

 > Case Presentation Top

A 32-year-old gentleman presented to our institute with the complaints of severe weight loss and prostration along with melena and abdominal pain for 10 months. A physical examination confirmed that his skin was extremely pale, although there was no icterus, clubbing, edema, or cyanosis. His vital signs were all within normal limits. Abdominal examination revealed no palpable mass. Laboratory examination documented low hemoglobin level (8.8 gm/dL; normal range: 11–17 gm/dL) and positive occult blood test. Peripheral blood smears showed microcytic hypochromic red blood cell morphology. Computed tomography scan [Figure 1]a and [Figure 1]b of the abdomen and pelvis showed a mild homogenously enhancing asymmetric intraluminal wall thickening of size 47 mm × 33 mm involving the proximal ileum. Multiple subcentimetric mesenteric and retroperitoneal lymph nodes were seen. The patient had undergone laparotomy and segmental resection of the ileum, and mesenteric lymph nodes were sent to pathology for further evaluation. Macroscopically, there was a polypoidal growth measuring 6.0 cm × 3.5 cm × 1.0 cm, protruding outside the ileum [Figure 1]c. The mass was solid, homogeneous, and grayish white in color. Microscopic examination revealed a tumor predominantly located in the submucosa involving the mucosa, as shown in [Figure 2]a and [Figure 2]b, muscularis propria, and serosa. The neoplastic cells were arranged in nesting, pseudoalveolar, as shown in [Figure 2]c and [Figure 2]d, and focally pseudopapillary pattern. There were numerous osteoclastic giant cells interspersed within the neoplastic population. The individual tumor cells were polygonal in shape with abundant clear cytoplasm, large vesicular round-to-oval nucleus, and inconspicuous-to-conspicuous nucleoli [Figure 2]e. Lymphoplasmacytic infiltration was noted. Mitosis was brisk (4–8/10 hpf). Focal necrosis was noted. The lymph nodes were not involved by the tumor. Immunohistochemically, the neoplastic cells were diffuse strong nucleocytopasmic positive for S-100 protein [Figure 3]a and [Figure 3]b. Markers for gastrointestinal stromal tumor (GIST), CD117, and DOG 1 were negative [Figure 3]c and [Figure 3]d. Melanocytic markers HMB 45 and melan-A [Figure 3]e and [Figure 3]f were also negative. Negative stain for pan-cytokeratin [Figure 3]g, smooth muscle actin [Figure 3]h, and desmin excluded the possibilities of carcinoma and leiomyosarcoma. The tumor cells were also negative for CD34 [Figure 3]i; hence, epithelioid angiosarcoma was ruled out. Ki-67 was positive in 10%–15% which indicates high proliferative activity [Figure 3]j. Fluorescence in situ hybridization (FISH) analysis for EWSR1 Break Apart Probe on paraffin-embedded tissue showed evidence of 22q12 rearrangement in 60 out of 100 (60%) interphase nuclei score. The average percent positive cells >15% (≥15/100) are considered the sample as positive. The CytoTest LSP EWSR1 5' CytoOrange/LSP EWSR1 3' CytoGreen probe was used, where native state of EWSR1 Break Apart Probe will be seen as two adjacent or fused (overlapping) red/green (yellow) signals [Figure 4]. However, EWSR1 gene rearrangement presented as one red and one green separated signal. The patient did not receive chemotherapy or radiotherapy. During follow-up, the patient remained disease-free for 20 months.
Figure 1: (a and b) CECT of the abdomen and pelvis shows an irregular hyperenhancing mass arising from the antimesentric border of distal small bowel with probable intraluminal extension. Fat planes with the rest of the bowel appears maintained. Mild perilesional fat stranding is seen. No significant mesenteric lymphadenopathy is seen. (c) Polypoidal mass measuring 6 cm × 3.5 cm × 1 cm projecting outside from the serosal aspect of ileum

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Figure 2: (a) Tumor is located in the submucosa (H and E, ×40). (b) Tumor infiltrating the mucosa and muscularis propria (H and E, ×40). (c) Tumor cells are arranged in nests (H and E, ×100). (d) Cells arranged in the alveolar pattern with numerous osteoclastic giant cells (H and E, ×100). (e) The cells are polygonal with vesicular nuclei and clear cytoplasm

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Figure 3: (a) Tumor cells show cytoplasmic and nuclear positivity for S-100 (IHC, ×40). (b) Tumor cells show cytoplasmic and nuclear positivity for S-100 and the osteoclastic giant cells are negative for the S-100 protein (IHC, ×400). (c) Tumor cells are negative for CD117 (IHC, ×100). (d) Tumor cells are negative for DOG1 (IHC, ×100). (e) Tumor cells show positivity for HMB45, (IHC, ×100). (f) Tumor cells are negative for melan A (IHC, ×100). (g) Tumor cells are negative for CK (IHC, ×100). (h) Tumor cells are negative for SMA (IHC, ×100). (i) Tumor cells are negative for CD34 (IHC, ×100). (j) Ki-67 index is 15% (IHC, ×400)

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Figure 4: Fluorescence in situ hybridization test using break-apart probe (EWSR1 22q12 translocation), 60% tumor cells showed positivity

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 > Review of World Literature and Collection Of Data Top

A systematic search of world literature on the malignant GNET, over the past two decades (January 1998–September 2019), was done by using search engines such as the PubMed, PubMed Central, Medline, and Directory of Open Access Journal databases. Following keywords were used for the literature search: clear cell sarcoma-like tumor of the gastrointestinal tract, clear cell sarcoma lacking melanocytic differentiation, malignant gastoneuroectodermal tumour, and neuroectodermal tumour with EWSR1 gene rearrangement. The references of all articles were cross-checked for full length articles. The tumors that showed melanocytic differentiation either by immunohistochemistry (IHC) or by electron microscopy were excluded from the study. Since 1998, 61 cases of GNET were described in 31 articles. The largest series of 16 cases were described by Stockman et al. in 2012. All the cases were reviewed in relation to clinical presentation, radiological findings, gross and microscopic findings, IHC, molecular genetics (wherever available), ultrastructural findings (wherever available), management, prognosis, and outcome.

 > Etiology Top

The etiology of GNETs is unknown. Six cases of GNETs document a past history of hepatoblastoma (2 cases), lymphoblastic leukemia (2 cases), neuroblastoma, and Ewing sarcoma, in early childhood, suggesting that genetic aberration in the embryonic stage or radiation exposure could be attributed as risk factors to the development of this tumor later in life.[6],[7],[8],[9],[10],[11] A single case was associated with immunoglobulin-G4-related sclerosing inflammation and another with intra-abdominal granulomatous inflammation, suggesting the possible role of immune factors in its pathogenesis.[12],[13] Absence of melanocytic markers and expression of SOX-10 protein in GNETs show that they originate from the neural crest precursor cells that are unable to differentiate into melanocytic lineage through uncertain mechanisms.[5] However, this entity is now defined with a pathognomonic translocation, harboring EWSR1-cyclic adenosine monophosphate responsive binding protein 1 (CREB1) or EWSR1-ATF1 gene fusion.

 > Clinical Presentation Top

Sixty-two cases of malignant GNET (including the present case) are described till date. The data show that it affects male and females equally, with male:female ratio = 1:1. The age ranged from 10 to 81 years. Nine cases were seen in the pediatric population.[5],[8],[10],[14],[15],[16],[17],[18] Their mean age at the time of disease manifestation was 14.7 years (range, 10–18 years). Similarly, the mean age at presentation among adults was 40 years (range, 20–81 years).

The most common site of involvement of GNET is the small intestine (72%). Ileum is the most common site (24 cases)[4],[5],[7],[8],[9],[10],[12],[13],[14],[19],[20],[21],[22],[23],[24],[25] followed by stomach in 12 cases[5],[7],[8],[11],[15],[16],[26],[27],[28] and jejunum in 12 cases.[5],[7],[8],[12],[17],[29],[30],[31] In seveb cases,[5],[6],[18],[22],[32] the exact site of involvement was not specified, though all these cases were located in the small bowel. Colon was rarely involved (4 cases)[4],[5],[33] by GNET. An isolated case of GNET occurring in the distal esophagus[34] and one involving both the small and large bowel[22] were also documented. Patients usually present with abdominal pain, with features of intestinal obstruction, and few show symptoms of unexplained anemia, melena, fever, and weight loss. Three cases had prior history of adenocarcinoma of colon,[4] renal cell carcinoma,[7] and seminoma.[19] At the time of diagnosis, locally advanced and metastatic cases were 38% and 26%, respectively. The common metastatic sites reported were liver, mesenteric nodes, peritoneum, and lung. Clinicopathological features of all the cases reported as GNETs are summarized in [Table 1].
Table 1: Gastrointestinal neuroectodermal tumor: A review of 62 cases (including the present one) reported worldwide (1998-2019)

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 > Imaging Characteristics Top

After a thorough review of literature, we could not find the characteristic features to diagnose GNET on imaging. Tumor location and extension of the tumor though can be detected, a diagnosis of GNET is difficult based on the radiology alone. Although histopathology is the gold standard for diagnosis of this entity, imaging has a significant role in determining the extent of the disease and to detect recurrence in the follow-up.

 > Pathologic Features Top

Macroscopic features

The tumor size ranged from 2.5 to 13.5 cm (mean, 5 cm). These tumors are centered in the wall of the GI tract. The tumor may present as a polypoidal mass protruding into the bowel lumen or a circumferential thickening of the bowel wall or as an ulceroproliferative growth with extension into the mucosa and serosa. On cut-section, they are usually solid, firm, tan white and show lobulations and necrosis.[5] Cystic changes are also noted.

Microscopic features

Tumor cells display a diverse pattern of arrangement from frequent nesting to pseudoalveolar, pseudopapillary, fascicular, and occasionally microcystic or rosette-like[5],[32] architecture. The individual cells are round-to-oval, epithelioid or spindle cells, with eosinophilic to clear cytoplasm. Oncocytic or granular changes were also noted.[28] The nuclei are centrally located with vesicular nuclei and may show prominent nucleoli. Few cases have documented nuclear pleomorphism.[22] Tumor giant cells are also described in a few studies. Nearly 50% of cases show scattered nonneoplastic osteoclastic giant cells which are invariably positive for CD68. Necrosis and frequent mitotic figures were also noted.[13],[17] None of the cases described, in the literature, showed melanin pigment either on hematoxylin and eosin section or on special stain, thus providing a clue for the diagnosis. Histopathological, ultrastructural, and molecular features of all the cases reported till date are shown in [Table 2].
Table 2: Histopathological, ultrastructural, and molecular features of 62 cases of gastrointestinal neuroectodermal tumor reported till date

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All the cases in this study were negative for melanospecific markers such as HMB-45, melan-A, and tyrosinase and displayed strong and diffuse nuclear and cytoplasmic positivity for S-100 protein. Few cases have documented variable positivity for at least one of the neuroendocrine markers[5],[13],[31] including synaptophysin, CD56, and neuron-specific enolase. Primitive neural crest cell marker SOX-10 was positive in 100% of cases where the stain was performed supporting its origin from neural crest lineage.[5],[28] Absence of these markers CD117, DOG-1, CD34, smooth muscle actin, desmin, and pan-cytokeratin supports a diagnosis of GNET. The proliferative marker Ki-67 showed nuclear positivity ranging from 5% to 30.4% in the present study.

Molecular genetics

Genetically, GNETs are characterized by EWSR1 (22q12.2) gene rearrangement in the majority of cases. In this study, 71% (44/62) cases had molecular analysis. In 52% of cases, EWSR1 gene rearrangement (23/44) was noted. The common fusion partners are ATF1 seen in 25% (11/44) and the CREB1 seen in 23% (10/44). Of the 16 cases described by Stockman et al., molecular study was done in 14 cases. Twelve cases showed EWSR1 gene rearrangement, and two cases were negative for EWSR1. Of the 12 cases positive for a split EWSR1 signal, six showed rearrangement of ATF1, three showed rearrangement of CREB1, two cases neither showed rearrangement with ATF1 and CREB1 genes, and in one case, ATF1 and CREB1 was not evaluated. Hence, Stockman et al. proposed that fused in sarcoma gene (FUS; 16p11.2) could be an alternative partner gene as it shares extensive nucleotide sequence homology with EWSR1. However, in their study, they did not find any case where there was a rearrangement of the FUS gene. Other tumors that share the EWSR1 gene rearrangement include Ewing sarcoma, desmoplastic small round cell tumor, myxoid liposarcoma, extraskeletal myxoid chondrosarcoma, angiomatoid fibrous histiocytoma, clear cell sarcoma, primary pulmonary myxoid sarcoma, myoepithelial neoplasm, and low-grade fibromyxoid sarcoma.[35] Therefore, EWSR1 gene rearrangement is not an absolute criterion for its diagnosis, and hence, a combination of morphology, IHC, and molecular study can help a pathologist in giving a correct diagnosis.

Ultrastructural findings

Electron microscopy studies have been reported in 16 cases of GNETs[5],[8],[9],[11],[13],[14],[22],[26] [Table 2]. The cells are polygonal with interdigitated and bulbous synaptic processes. No melanosomes or melanosome-like structures identified, although rare premelansomes were identified in three cases.[14],[19],[26] Stockman et al. identified dense core secretory granules which was consistent with neuroendocrine differentiation and hence labeled this entity as GNET.

Differential diagnosis

The mimickers of GNET are GIST, CCS-GI, poorly differentiated adenocarcinoma, neuroendocrine tumor, primary or metastatic melanoma, epithelioid malignant peripheral nerve sheath tumor (MPNST), and malignant granular cell tumor.

GISTs macroscopically and microscopically resemble GNETs. It is imperative to distinguish this entity from GIST because of their different pathogenesis and response to treatment. Both of them can show epithelioid or spindle cell morphology in sheets and fascicles. Osteoclast-like giant cells are commonly found in GNETs. Ninety percent of GIST expresses CD117, DOG1, and CD34 markers, whereas GNET is usually negative for these markers.[36]

CCS typically occurs in soft tissue and involvement of the GI tract is rare. Although the two tumors resemble morphologically, immunohistochemically and ultrastructurally they are different. CCS harbors tumor-derived giant cells rather than osteoclastic-like giant cells, expresses melanocytic markers, such as HMB-45 and melan A, and at the ultrastructural level shows numerous melanosomes at different levels of maturation, thus differentiating from GNET.

Poorly differentiated adenocarcinoma has a nested and sheet pattern of arrangement of tumor cells, causing a diagnostic dilemma. However, diffuse positivity for cytokeratin helps clinch a correct diagnosis. Although GNET expresses neuroendocrine markers, the presence of OLGC and the EWSR1 rearrangement differentiates it from a neuroendocrine tumor.

Metastastic melanoma, another close differential of GNET, usually has a prior history of cutaneous involvement. In addition ,presence of melanin pigments, expression of melanocytic markers and lack of EWSR1 rearrangement are important clues in differentiating both the lesions.

Epithelioid MPNST usually arises from a nerve or a preexisting neurofibroma or schwannoma and comprises polygonal cells with a prominent nucleolus, often merging with the spindled areas. It lacks osteoclastic giant cells and is strongly and diffusely positive for S-100 in contrast to GNET which shows patchier staining.[37]

Malignant granular cell tumors are rare in these sites; however, genetic analysis is valuable in distinguishing the two entities.[37] Detailed morphology, IHC, and differential diagnosis of GNETs are shown in [Table 3].
Table 3: Immunohistochemistry and differential diagnosis of malignant gastrointestinal neuroectodermal tumor

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 > Treatment Top

Surgical excision is the mainstay treatment. At present, the treatment offered is surgical excision of the involved bowel segment, followed by regular image monitoring for recurrence and metastasis.[38] Although there is no consensus for chemotherapy after the surgical excision, three patients received chemotherapy.[11],[13],[24]

 > Survival and Prognostic Factors Top

The clinical behavior of gastrointestinal CCS is aggressive, independent of the fusion type.[4] The tumor has a worse prognosis than its soft tissue counterpart. Adverse prognostic factors identified are larger tumor size and microscopic tumor necrosis.[4] There is a propensity to metastasize to the liver and/or regional lymph nodes at the time of initial presentation or as recurrence[4],[8] Although most patients developed liver metastases, in a few cases, intraperitoneal spread was noted.[4],[7],[8] The tumor behaves more aggressively in the pediatric population compared to adults. In this study, we encountered nine pediatric GNETs where 67% of cases showed a tumor size of 5 cm or more. The time to distant recurrence was variable, ranging from 2 weeks to 109 months. The follow-up period for all these cases ranged from 2 to 161 months.

 > Conclusion Top

GNET is an extremely rare malignant tumor of GI tract and can be mistaken for other mesenchymal tumors that occur in the GI tract. A tumor arising from the wall of GI tract, with epithelioid to spindle morphology, diffusely positive for S-100 protein, and lacking melanocytic differentiation on IHC should prompt a pathologist to consider GNET in the differential diagnosis. Awareness of this entity is equally important for clinicians and pathologists to avoid delay in diagnosis and management. A multimodality approach, assessing the clinical, gross characteristics, histopathological features, IHC, and genetic and sometimes ultrastructural findings is helpful for rendering a correct diagnosis.

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Conflicts of interest

There are no conflicts of interest.

 > References Top

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Ekfors TO, Kujari H, Isomäki M. Clear cell sarcoma of tendons and aponeuroses (malignant melanoma of soft parts) in the duodenum: The first visceral case. Histopathology 1993;22:255-9.  Back to cited text no. 2
Bridge JA, Borek DA, Neff JR, Huntrakoon M. Chromosomal abnormalities in clear cell sarcoma. Implications for histogenesis. Am J Clin Pathol 1990;93:26-31.  Back to cited text no. 3
Antonescu CR, Nafa K, Segal NH, Dal Cin P, Ladanyi M. EWS-CREB1: A recurrent variant fusion in clear cell sarcoma--Association with gastrointestinal location and absence of melanocytic differentiation. Clin Cancer Res 2006;12:5356-62.  Back to cited text no. 4
Stockman DL, Miettinen M, Suster S, Spagnolo D, Dominguez-Malagon H, Hornick JL, et al. Malignant gastrointestinal neuroectodermal tumor: Clinicopathologic, immunohistochemical, ultrastructural, and molecular analysis of 16 cases with a reappraisal of clear cell sarcoma-like tumors of the gastrointestinal tract. Am J Surg Pathol 2012;36:857-68.  Back to cited text no. 5
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1], [Table 2], [Table 3]


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  >Abstract>Introduction>Case Presentation>Review of World ...>Etiology>Clinical Present...>Imaging Characte...>Pathologic Features>Treatment>Survival and Pro...>Conclusion>Article Figures>Article Tables
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