Primary rare anaplastic large cell lymphoma, ALK positive in small intestine: case report and review of the literature

Abstract

BackgroundPrimary anaplastic large cell lymphoma, ALK positive in small intestine is clinically rare and the clinical, radiological and pathological information are generally not available. Here, we report a case of 32-year-old male with ALK positive anaplastic large cell lymphoma at the junction of jejunum and ileum, and highlight the clinicopathological features and the differential diagnosis of this type lymphoma.Case presentationThe patient presented with right middle abdominal mass for 1 month with sporadic pain. Computed tomography (CT) showed a mass measured 8.5 × 7.4 × 4 cm at the junction of jejunum and ileum. The diagnosis was made after pathological examination of the excised tissue by enterectomy. Grossly, the mass was located predominately in intestinal wall with grayish appearance and blurry boundary. Microscopically, almost all layers of the intestinal wall were infiltrated by pleomorphic tumor cells with diffuse and cohesive growth pattern. The neoplastic cells were mainly medium to large size with moderate basophilic cytoplasm. Most of them had hyperchromatic nuclei and prominent nucleoli. “Hallmark” cells were easily detected. Immunohistochemically, tumor cells are characterized by CD30, ALK, CD5, TIA-1, Granzyme B, EMA positive staining, and CD2, CD3, CD7, CD4, CD8, CD20, CD79a negative staining. The Epstein-Barr virus encoded RNAs (EBERs) genome was also negative. A diagnosis as primary small intestinal ALK positive anaplastic large cell lymphoma was finally made. The patient received CHOP chemotherapy and is alive till now without recurrence 5 months after enterectomy.ConclusionsPrimary small intestinal ALK positive anaplastic large cell lymphoma is rare. The accurate diagnosis should be based on combined consideration of clinical characteristics, CT image and pathological features, and should be distinguished from other lymphomas or solid tumors in small intestine.

The second-generation ALK inhibitor alectinib effectively induces apoptosis in human neuroblastoma cells and inhibits tumor growth in a TH-MYCN transgenic neuroblastoma mouse model

“…In addition, ALK -activating point mutations have been identified in approximately 8% of investigated NB tumors [1]. In prior studies, inhibition of ALK led to a significant decrease in cell proliferation in ALK -positive cancers, including non-small cell lung cancer (NSCLC) [26, 27, 29], anaplastic large cell lymphoma (ALCL) [6, 34] and NB [15, 44, 50, 52]. ALK-targeted chemotherapies have been shown to downregulate PI3K/Akt signaling, leading to cell apoptosis and tumor regression [4, 15, 51, 52].…”

ALK-positive anaplastic large cell lymphoma presenting multiple lymphomatous polyposis: A case report and literature review

“…According to a recent review on gastrointestinal ALCL, the rate of ALK expression among gastrointestinal ALCL was low at 24%[11]. As far as we can determine, ALK+ ALCL of the gut has been reported in only eleven cases[11-20]. The clinical features are summarized in Table 2 for a total of 12 gastrointestinal ALK+ ALCL cases, including our patient.…”

“…Of these, four cases were reported to involve the esophagus[12-15], all showing fungating tumors. Of the remaining cases, two involved the stomach[11,16], one involved the duodenum[17], one involved both the stomach and duodenum[18], and three involved the small intestine (jejunum/ileum)[11,19,20]. Only three of these last seven cases showed macroscopic findings by resection or endoscopy; the morphology showed a mass formation pattern, such as a submucosal or bulky tumor.…”

Undifferentiated large cell/rhabdoid carcinoma presenting in the intestines of patients with concurrent or recent non-small cell lung cancer (NSCLC): clinicopathologic and molecular analysis of 14 cases indicates an unusual pattern of dedifferentiated metastases

“…Considering the third point, undifferentiated metastatic NSCLC should be distinguished from few other entities including in particular rhabdoid gastrointestinal stromal tumor (GIST), anaplastic large cell lymphoma (ALCL), and epithelioid inflammatory myofibroblastic sarcoma [27][28][29]. The detection of a conventional tumor component and/or Fig.…”

“…All of our 11 cases tested lacked mutations in these two genes, thus largely ruling out the possibility of dedifferentiated GIST. Anaplastic large cell lymphoma may closely mimic undifferentiated rhabdoid carcinoma and strongly express EMA [28]. Homogeneous expression of CD30 and cytotoxic markers and also of ALK in the majority of cases is diagnostic and rules out this consideration.…”