| Abstract|| |
Background: Follicular dendritic cell sarcoma (FDCS) is a rare tumor derived from follicular dendritic cells (FDC) occurring in lymph nodes and extranodal sites. It is usually regarded as an indolent tumor with a tendency of local recurrence but a low risk of metastasis. Common extranodal sites are liver, lung, tonsil, spleen, soft tissue, and mediastinum. Extranodal FDCS of gastrointestinal tract (GIT) is exceedingly rare, with just 36 cases reported in the literature. Methods: We report an unusual case of FDCS of caecum in a 13-year-old boy who presented as intussuception. On histology, it posed a diagnostic challenge for us. An inconclusive initial immunohistochemistry (IHC) lead us to suspect FDCS which was confirmed by FDC markers. Conclusions: The diagnosis of FDCS at extranodal site like GIT is all the more challenging because of its rarity, morphologic heterogeneity, and lack of awareness.
Keywords: Extranodal, follicular dendritic cell sarcoma, follicular dendritic cells, gastrointestinal
|How to cite this article:|
Jagdale RV, Pol JN. Follicular Dendritic cell sarcoma of caecum in a young boy presenting as Intussusception. Indian J Pathol Microbiol 2021;64:759-62
|How to cite this URL:|
Jagdale RV, Pol JN. Follicular Dendritic cell sarcoma of caecum in a young boy presenting as Intussusception. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 Dec 7];64:759-62. Available from: https://www.ijpmonline.org/text.asp?2021/64/4/759/328572
| Introduction|| |
Follicular dendritic cell sarcoma (FDCS) is a rare tumor derived from the follicular dendritic cells (FDCs). FDCS presents with lymph node (LN) disease in 31%, extranodal disease in 58%, and both nodal and extranodal disease in 10% of cases. A variety of extranodal sites can be affected commonest include liver, lung, tonsil, spleen, soft tissue, mediastinum, and gastrointestinal tract (GIT). Hollowood et al. first reported extranodal FDCS (EFDCS) of GIT in 1995. Till date, 36 cases of EFDCS of GIT have been described in English Literature.,,,,,,,,,,,, We describe FDCS of caecum, in a 13-year-old boy who presented as intusussception.
| Case Report|| |
A 13 year boy underwent ileocaecal resection for intussusception. Grossly, the resection of terminal ileum and caecum measured 12 cm in length. The caecum showed a grayish white, fleshy mass 6 × 5 × 4 cm infiltrating the full thickness of caecal wall. Five mesenteric LNs were dissected, largest measuring 3 × 3 × 2 cm. Microscopically, the caecal wall showed a submucosal mass with morphologic heterogeneity composed of sheets, nodules, whorls, and fascicles of spindle to epithelioid cells with oval vesicular nuclei and distinct nucleoli. Nuclear atypia, pseudoinclusions, multinucleation with occasional wreath-like nuclei were noted. Occasional mitosis and focal necrosis was seen. The stroma showed a polymorphous infiltrate comprising of lymphocytes, plasma cells, histiocytes, and eosinophils. The sections from LNs revealed tumor metastasis of similar morphology [Figure 1] and [Figure 2]. Considering the young age and histology, the possibilities considered were anaplastic large cell lymphoma (ALCL), Langerhan cell histiocytosis (LCH), gastrointestinal stromal tumor (GIST), and inflammatory myofibroblastic tumor (IMT). The primary immunohistochemistry (IHC) panel comprised of LCA, CD3, CD20, CD30, ALK1, CD1a, CD34, CD117, and DOG1, all of which were negative. The other differential diagnoses which needed exclusion were epithelioid leiomyosarcoma, poorly differentiated carcinoma, and melanoma. Hence, a second panel comprising of Vimentin, SMA, Desmin, CK, EMA, and S100 was placed [Figure 3] and [Figure 4]. With just Vimentin positivity and a striking morphologic feature of sprinkled lymphocytes amidst spindle and epithelioid cells, a possibility of FDCS needed to be considered. So, a tertiary panel of FDC-specific markers CD21, CD23, and CD35 was put, all of which were strongly expressed [Figure 5]. Hence, a diagnosis of EFDCS of caecum with metastasis to regional LNs was made.
|Figure 1: H and E sections show (a) submucosal tumor in the caecal wall (×40), (b) dissecting the muscle coat (×100), (c) composed of spindle cells (×400), and (d) epithelioid cells with admixed lymphocytes (×400)|
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|Figure 2: H and E sections show (a) nuclear atypia and occasional wreath like multinucleated giant cell mimicking ALCL (×400), (b) increased mitoses (arrows) (×400), (c) sprinkling of lymphocytes amidst tumor cells, occasional pseudoinclusion (arrow) (×400), (d) mesenteric LN metastasis (×40)|
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|Figure 3: IHC Tumor cells are negative for (a) SMA (b) Desmin (c) CD117 (d) CD34 (e) CK (f) EMA (g) S100 and (h) DOG1. (×100)|
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|Figure 4: IHC Tumor cells are negative for (a) LCA (b) CD20 (c) CD3 (d) CD30 (e) ALK1 and (f) CD1a. (×400)|
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|Figure 5: IHC- Tumor cells express (a) CD21 (b) CD23, (c) CD35 and (d) Vimentin. (×100)|
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| Discussion|| |
FDCs are antigen presenting cells of the primary and secondary follicles. They are present in the LNs as well as extranodal sites, either as acquired lymphoid tissue or as part of the organized constitutive lymphoid tissue. Tumor arising from FDCs is called FDCS. As FDCs are present in the nodal and extranodal sites, FDCS can arise at both nodal and extranodal locations. Common extranodal sites are liver, lung, tonsil, spleen, soft tissue, mediastinum, GIT, retroperitoneum, and mesentry.
Hollowood et al. reported the first case of extranodal FDCS (EFDCS) of the GIT in 1995, since then 36 cases of FDCS of GIT have been reported in the world literature.,,,,,,,,,,,, Saygin et al. reviewed all 462 cases of dendritic cell sarcoma reported in the literature till 2013, of which 343 were FDCS and 18 were EFDCS of GIT. Hassan et al. have published an exclusive case series of eight cases of EFDCS of the GIT. Additionally, there are 10 single case reports from 2013 to 2020.,,,,,,,,, Four single case reports are from India. [3, 4, 6, 13]
FDCS is defined by “WHO” as “a neoplastic proliferation of spindle to ovoid cells showing morphological and immunological phenotype features of FDCs.” There is a wide patient age range with an adult predominance (medium age; 50 years) and equal sex distribution.
Etiopathogenesis of FDCS remains unclear. Castleman disease has been suggested as a precursor lesion. EBV is involved in the pathogenesis of a small subset of cases, but most of them were reported as “Inflammatory pseudotumor-like FDCS.” Many reports suggest an association with autoimmunity.
Most of the patients with FDCS of GIT present as abdominal pain and weight loss. Some may have constipation and epigastric pain. The tumors are often large with a mean size of 7 cm. Most patients have localized disease at presentation. Distant metastasis is rare and LNs, lung, and liver are commonest sites of metastasis.
The neoplasm typically consists of spindled to ovoid cells forming fascicles, storiform arrays, whorls, diffuse sheets, or vague nodules. The individual cells show indistinct cell borders and moderate amount of eosinophilic cytoplasm. The nuclei are oval or elongated with vesicular chromatin and small but distinct nucleoli. The mitotic rate is usually 0–10 mitoses per 10 high power fields depending on the grade of the tumor. The tumor shows distinct sprinkling of small lymphocytes. Variations in morphology include epithelioid features, presence of coagulative necrosis, giant cells, and areas harboring marked atypia. Lan Li et al. categorized FDCS into low-grade and high-grade groups based on four major parameters including architectural pattern, cellular atypia, mitotic activity, and Ki67 labelling index. They also proposed a model for recurrence risk assessment based on tumor size (≥5 cm) and histologic grade, and classified them into low-, intermediate- and high-risk groups. The recurrence rates were 16%, 56%, and 73% and mortality was 0%, 4%, and 45%, respectively. With large size (6 cm) and high grade histology, our case was categorized into high risk group. FDCS is consistently positive for one or more FDC specific markers, CD21, CD35, and CD23. It is also commonly positive for CXCL13, Podoplanin, Clusterin, Vimentin, and Fascin and variably positive for EMA, S100, and CD68.
EFDCS of GIT are often not considered in the differential diagnosis of the spindle and epithelioid tumors because of lack of awareness. Important differential diagnoses are GIST, smooth muscle tumors, neural tumors, lymphomas, and in presence of significant nuclear atypia – sarcomatoid and undifferentiated carcinoma and undifferentiated sarcoma. Inflammatory pseudotumor-like FDCS is usually mistaken for IMT. Since this was a 13 year boy with morphologic overlap, possibilities of LCH and ALCL were also considered. However, all the relevant IHC markers were negative, except Vimentin. On reviewing the morphology, the distinct sprinkling of lymphocytes was a clue to FDCS which was confirmed by IHC.
After revisiting the literature, we realized some unique and noteworthy features in our case. This is the third youngest case of FDCS in the literature; the youngest being 7 years and 9 years old. This is the youngest case of FDCS reported at an extranodal site. Overall, only four cases of FDCS of caecum have been documented.,, Interestingly, our case presented with intussusception which too is exceptionally rare, with only two prior cases reported.,
Low-grade and high-grade FDCS behave like and are treated as low-grade and high-grade soft tissue sarcomas. Radical surgery is the mainstay of treatment. Results of adjuvant chemo-radiation are diverse and usually reserved for locally advanced and metastatic disease.
This patient received postoperative adjuvant chemo-radiation since he had regional LN metastasis. He succumbed to the disease 3 years after the diagnosis. As a whole, EFDCS are more aggressive and the intra-abdominal location in an additional adverse factor. Our patient falls into high-risk group. This explains the rapid progression in our case, although FDCS otherwise are quite indolent.
To conclude, because of dearth of published literature, FDCS is often under-reported, particularly at extranodal sites. Hence, we reiterate that pathologist must think of FDCS even at extranodal sites, especially if it shows typical spindle and/or epithelioid morphology, with sprinkling of lymphocytes.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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Rakhi V Jagdale
Department of Pathology, Shri Siddhivinayak Ganpati Cancer Hospital, Miraj, Sangli - 416 410, Maharashtra
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]