Histiocytic/dendritic cell sarcoma arising from follicular lymphoma involving the bone: a case report and review of literature

Histiocytic/dendritic cell sarcomas arising from follicular lymphoma are very rare and poorly understood lesions. We describe a case, which is unique in that it presented with a hipbone lesion simultaneously with axillary lymphadenopathy. Biopsy of the axillary lymph node showed a low-grade follicular lymphoma. The hipbone lesion was comprised two cell populations, one representing diffuse large B cell lymphoma and the other a histiocytic/dendritic sarcoma. The cells of all three lesions contained an IGH/BCL2 rearrangement, suggesting that both diffuse large B cell lymphoma and histiocytic/dendritic sarcoma differentiation developed from the same low grade precursor (follicular lymphoma). This case illustrates that sarcomatous transdifferentiation of follicular lymphoma can be an unpredictable local phenomenon and that it can occur extra nodally in the bone marrow. It may also occur concurrently with the progression of follicular lymphoma to a diffuse large B cell lymphoma.     

胃转移性恶性黑色素瘤1例

目的:探讨胃转移性恶性黑色素瘤的临床病理特征、鉴别诊断及治疗与预后.方法:报道1例胃转移性恶性黑色素瘤, 取胃活检经HE染色及免疫组织化学染色诊断. 并复习国内外相关文献.结果:活检组织切片光镜下见胃黏膜固有层弥漫浸润的小细胞, 部分呈巢状排列, 细胞胞质较丰富, 核偏位, 核仁大而明显, 仅少部分细胞的胞质内可见黑色素颗粒. 免疫组织化学染色显示vimentin(+), S-100(+), HMB45(+),AE1/AE3(-). 病理诊断为胃(底、体交界)恶性黑色素瘤, 结合病史考虑为转移性.结论:恶性黑色素瘤转移到胃时没有典型临床表现, 若出现与原发肿瘤不相关的症状, 要高度怀疑转移. 主要与胃原发性肿瘤鉴别. 预后差. 对远处转移的最初部位为胃的患者, 进行根治性手术能有效缓解症状, 并可能对延长生存期有帮助.     

Intra-muscular follicular dendritic cell sarcoma in the thigh: A case report

Rationale:Follicular dendritic cell sarcoma (FDCS) is an intermediate-grade malignancy originating from follicular dendritic cells. Nodal FDCS is the most common type, meaning that the extranodal type may not be recognized and could be easily misdiagnosed. Reported extranodal sites include the head and neck, retroperitoneum, spleen, liver, and gastrointestinal tract. FDCS in the soft tissue is extremely rare.Patient concerns:A 75-year-old male presented with complaints of a localized swelling and intra-muscular soft tissue mass in the left upper thigh.Diagnosis:The present tumor consisted of fascicular or vague storiform-arranged spindle cells with less pleomorphism and many lymphoid aggregates. Tumor cells were positive for CD21, CD35, CD68, vimentin, and EGFR. Intra-muscular FDCS was confirmed by immunohistochemical studies.Interventions:The patient received a wide marginal excision, followed by adjuvant radiotherapy.Outcomes:Symptomatic improvements were achieved and no subsequent relapses were observed.Lessons:If the tumor arises in the extranodal sites, especially in the soft tissue, it is difficult to include FDCS in the differential diagnosis. When the immunoprofile is not consistent with that of common spindle cell tumors, immunostaining for follicular dendritic cell markers such as CD21, CD23, and CD35, as well as further immunohistochemistry for D2-40, CD68, EGFR, Epstein-Barr virus, and BRAF can be helpful for the diagnosis and subtyping of FDCS. To the best of our knowledge, the present case is the first case of intramuscular FDCS.     

Cutaneous langerhans cell sarcoma: a case report and review of the literature

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells that occurs in lymph nodes, liver, skin, spleen, lung, and bone. We report a case of LCS in a 47-year-old man with a 6-month history of scalp mass and cervical lymphadenopathy. Clinical and pathologic data were available. A histologic examination demonstrated a proliferation of cells with malignant cytologic features. Because of its poorly differentiated morphologic features, hematologic and nonhematologic entities were ruled out by immunohistochemical screening with a broad panel of antibodies. Ultrastructural studies demonstrating Birbeck granules and consistent expression of CD1a, S-100 protein, and langerin by immunohistochemistry were helpful in identifying the Langerhans cell origin.     

Follicular dendritic cell sarcoma: a report of 14 cases and a review of the literature

Follicular dendritic cell sarcomas (FDCS) are grouped with the histiocytic and dendritic cell neoplasms. The natural history and response to different treatments have not been well established. The cases of 14 patients with FDCS who were seen at M. D. Anderson between 1995 and 2005 were reviewed. Median patient age was 48 years (range, 25-69 years). Histologically, four cases showed low-grade features, three cases showed low-grade features with focal high-grade features, and five cases showed high-grade features. Tumors were positive for CD21, CD23, and CD35 in 83, 90, and 44% of cases, respectively. Twelve (92%) of 13 tumors were strongly positive for epidermal growth factor receptor. Information on initial treatment was available in 11 patients, which included surgery alone in one patient, surgery and radiation in two, surgery and chemotherapy in one, chemotherapy alone in three, chemotherapy and radiation in one, surgery followed by radiation and chemotherapy in three patients. In eight patients the initial chemotherapy regimen was cyclophosphamide, doxorubicin, vincristine, and prednisone. Complete remission was achieved in 7 (63%) of 11 patients. Ten patients were alive at a median follow up of 22 months, 3 (23%) of 13 had no evidence of disease, and 7 (53%) of 13 patients were alive with disease. Follicular sarcoma is an aggressive neoplasm. Although most of the patients initially responded to treatment, the majority of them (81%) relapsed. A better understanding of the biology of FDCS could guide our efforts in the development of new treatment modalities for this rare disease.     

Computed tomography and 18F-fluoro-2-deoxyglucose positron emission tomography findings of primary pulmonary follicular dendritic cell sarcoma: case report and a literature review

We report herein computed tomography and F-fluoro-2-deoxyglucose positron emission tomography findings of primary pulmonary follicular dendritic cell sarcoma in a 51-year-old man, which is an extremely rare malignancy originating from follicular dendritic cells. Computed tomography scan revealed a 2.4-cm, well-defined solitary pulmonary nodule with delayed contrast enhancement but without regional lymphadenopathy, distant metastasis, or calcification within the nodule. On F-fluoro-2-deoxyglucose positron emission tomography scan, this nodule showed moderate hypermetabolic activity with a maximum standardized uptake value of 3.2.     

Granular cell tumor of stomach： A case report and review of literature

Granular cell tumor (GCT) was described for the first time by Abrikosoff in 1926. It is a relatively rare neoplasm that may occur at many sites, but most commonly in the skin or soft tissues. The occurrence of GCT in the gastrointestinal tract is rare, accounting approximately for 8% of all tumors, among which the most common site is the esophagus, whereas gastric localization is very rare. Gastric GCTs can be solitary or, more frequently, associated with other gastrointestinal localization. Although GCTs are usually clinically and histologically benign, some malignant cases have been reported. Histologically, these tumors consist of polygonal and fusiform cells disposed in compact "nests" and immunohistochemical staining for S-100 protein supports the proposed derivation from Schwann cells. A correct preoperative diagnosis of this tumor can only be made in 50% of all patients and it is always based on endoscopic biopsy. Laparoscopic or conventional wedge resection represents the treatment of choice. In this study, the authors reported a case of a 49-year-old woman with a solitary granular cell tumor of the stomach with infiltrative pattern, successfully treated with surgical resection. A review of literature is also presented with emphasis on diagnostic criteria concerning the malignant form.     

Synchronous histiocytic sarcoma and diffuse large B cell lymphoma involving the stomach: a case report and review of the literature

We present a 62-year-old Chinese woman with histiocytic sarcoma (HS) and diffuse large B cell lymphoma (DLBL) located in the stomach. HS showed abundant eosinophilic cytoplasm with large, pleomorphic nuclei. Tumor giant and multinucleated cells were also prominent. Hemophagocytosis was common in the neoplastic cells. These cells were positive for CD68, CD45RO, and leukocyte common antigen (LCA), partly positive for S-100 and lysozyme, and negative for CD3, CD5, CD20, Pax-5, CD79a, CD1a, CD21, CD35, CD34, myeloperoxidase, HMB45 and cytokeratin (CK). These cells invaded the submucosa, muscularis mucosa, and serosa. DLBL was located in the mucosa and engulfed the covering epithelium and gland. The nuclei of tumor cells were medium-to-large in size, being ≥twice the size of normal lymphocytes. They were diffusely positive for CD20, Pax-5, and LCA, partly positive for vimentin, CD79a, CD10, CD30, CD15, and epithelial membrane antigen, and negative for S-100, CD1a, CD21, CD5, cyclin D1, CD23, CD35, HMB45, and CD68. Both types of tumor cell were positive for Oct-2, Bcl-2, and Bcl-6. These results suggest that HS may have shared a common clonal origin with DLBL, or may have transdifferentiated from DLBL The patient refused further chemotherapy, and died 7 months after surgical resection.     

母细胞性浆细胞样树突细胞肿瘤1例及文献复习

目的:通过病例报道及文献复习提高对母细胞性浆细胞样树突细胞肿瘤的临床及病理特征的认识。方法:报道1例母细胞性浆细胞样树突细胞肿瘤患者的临床表现、实验室检查及病理特点,观察CHOP方案疗效,并进行文献复习。结果:CHOP方案化疗对该患者疗效不佳。结论:母细胞性浆细胞样树突细胞肿瘤具有高度侵袭性,预后差,治疗方案尚未统一,尤其高龄患者的有效治疗还有待进一步的研究。     

Interdigitating dendritic cell sarcoma presenting simultaneously with acute myelomonocytic leukemia: report of a rare case and literature review

Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare tumor derived from interdigitating dendritic cells. We report the first case of a 64-year-old Chinese woman who was diagnosed with simultaneous IDCS and acute myelomonocytic leukemia (AML-M4). The patient had undergone chemotherapy for breast cancer 6 years previously. Based on the laboratory results, both the IDCS and the AML-M4 in this patient were determined to be of myelogenous origination. Furthermore, a review of 62 IDCS cases (Medline database, key word: IDCS) reported to date revealed that as many as 17 % of the patients had malignant disease and received radiotherapy and/or chemotherapy prior to developing IDCS, and that this group of patients showed worse prognosis compared with counterparts. The patient in the present report showed poor response to four cycles of sequential chemotherapy, and died 6 months after the initial diagnosis.     

Histiocytic sarcoma: a case report and review of the literature

Histiocytic sarcoma is a rare neoplasm, and its aetiology is unknown. It is a malignant proliferation of neoplastic cells showing immunophenotypic and morphologic features similar to tissue histiocytes. The clinical course of histiocytic sarcoma is usually agressive. The signs and symptoms of histiocytic sarcoma are systemic symptoms (fever, weight loss), hepatosplenomegaly, adenopathies, intestinal obstruction, rash and pancytopenia. We present the case of a 75 years old woman, with fever, weight loss, anorexia, fatigue, splenomegaly and pancytopenia. Bone marrow examination showed the diagnosis of histiocytic sarcoma.     

Hepatic inflammatory pseudotumor-like follicular dendritic cell tumor with hepatic lymphoma history: A case report and literature review

Rationale:Hepatic inflammatory pseudotumor (IPT)-like follicular dendritic cell (FDC) sarcoma is a very rare disease. Till now, only 19 cases were reported in the English literature. However, the coexistence of IPT-like sarcoma and non-Hodgkin lymphoma (NHL) in the same patient has never been reported. In this report, we presented a case of hepatic IPT-like FDC with hepatic NHL history of which both were successfully resected.Patient concerns:We reported a case of a 47-year-old male patient who presented with right upper abdominal discomfort. Nineteen years ago, he underwent liver resection of segment VII for hepatic NHL (B-cell lymphoma). He had a history of chronic hepatitis B virus infection. Serum alpha fetoprotein level was normal. However, imaging studies revealed a well-circumscribed, solid mass in the right hepatic lobe, he came back to the clinic because he was worried about a recurrence of the B-cell lymphoma.Diagnoses:Based on the patient''s past medical history and magnetic resonance imaging results, and he was diagnosed as hepatocellular carcinoma or hepatic NHL preoperatively.Interventions:Right hemi-hepatectomy was performed on this patient.Outcomes:Histological report showed features of a mixture of chronic inflammatory cells and variable amounts of spindle cells. Also, immuno-histo-chemical studies demonstrated that all the tumor cells showed strong nuclear in situ labeling for EBV-encoded small RNAs and strongly positive stainings with CD21 and CD35. The patient tolerated the surgery well, recovered smoothly and he was discharged on postoperative day 7 (day 7). The patient is still disease free after a follow-up of over 50 months.Conclusions:To our knowledge, this is the first report demonstrating hepatic IPT-like FDC sarcoma in a patient with primary hepatic NHL history. In regards to treatment, complete surgical resection should be performed and would acquire excellent long-term outcomes.     

Basaloid follicular hamartoma: a case report and review of the literature

Basaloid follicular hamartoma (BFH) is a rare, benign, skin adnexal tumor. Several clinical patterns have been reported, but they all share the same histopathological features. BFH may be hereditary or nonhereditary and can be accompanied by systemic diseases. Microscopic examination of BFH shows branching cords and anastomosing strands of basaloid cells in a loose, fibrous stroma. The most important pathological differential diagnosis is infundibulocystic basal cell carcinoma. These two lesions must be differentiated carefully based on clinical presentation and histopathological picture, and even molecular studies may be needed. We present a report of a 78-year-old woman with a solitary, asymptomatic, slow-growing skin tumor on her left scalp. No associated systemic disorders were found. On the basis of an excisional biopsy performed on the tumor, a pathological diagnosis of sporadic BFH was made.     

Ovarian follicular lymphoma: a case report and review of the literature

Primary ovarian lymphoma is extremely rare, and such a case is reported here. The patient was a 54-year-old Japanese female with abnormal genital bleeding. Pelvic CT and MRI showed a right ovarian tumor with a diameter of 7 cm and an irregular border. With the diagnosis of a right ovarian tumor, the patient underwent a simple hysterectomy and bilateral salpingo-oophorectomy. Microscopic examination of the right ovarian tumor revealed vaguely nodular growth of small lymphoid cells. They were CD10+, Bcl-2+, Cyclin D1- and CD21-, although CD21+ follicular dendritic cell clusters were present as a background component in each vague nodule. A conventional cytogenetic analysis revealed t(14;18)(q32;q21), and somatic hypermutation of the variable region of the immunoglobulin heavy chain gene (IgH) was confirmed by direct sequencing of subcloned DNA samples derived from PCR amplicons. These findings led us to characterize the lesion as follicular lymphoma, grade 1. The patient was free of detectable disease 9 months after initiation of post-surgical chemotherapy. Since the prognosis for primary ovarian lymphoma is relatively favorable in many cases, it is important to establish therapeutic methods for the cure of this disease using chemotherapy and radiotherapy without radical surgery.     

Primary squamous cell carcinoma of the stomach: a case report and review of the literature

Primary squamous cell carcinoma of the stomach is extremely rare. To date, only 80 cases have been reported. A 65-year-old man with complaints of epigastric pain and cachexia for the past year is presented. He had a tumour with infiltration of the corpus and antrum of the stomach. The tumour was unresectable, and the patient died within 3 months.     

Multidisciplinary diagnosis and treatment of recurrent follicular dendritic cell sarcoma in abdomen: A case report

Rationale:Follicular dendritic cell sarcoma (FDCS) is a rare malignant tumor derived from follicular dendritic cells, and is often associated with Castleman disease. Here we present a rare case of paraneoplastic pemphigus (PNP) with FDCS which required multidisciplinary approach for the diagnosis and treatment.Patient concerns:A 28-year-old Chinese female had FDCS recurrence, and primary clinical manifestation was PNP.Diagnoses:PNP with FDCS.Interventions:The patient received gamma globulin infusion, took anlotinib, and underwent plasma exchange therapy.Outcomes:The skin lesions recovered and there was no evidence of tumor recurrence.Lessons:The diagnosis and management of PNP with FDCS require close cooperation among surgeons, dermatologists, hematologists, otolaryngologists, oncologists, radiologists, pathologists, and respiratory doctors. The interesting clinical manifestations of this patient provide a multifaceted approach to the investigation of the interactions among FDCS, Castleman disease, and PNP.     

Left atrial spindle cell sarcoma: A case report and literature review

Rationale:Cardiac primary spindle cell sarcoma is 1 of the rarest cardiac malignancies, with only a few cases reported so far. Herein, we reported a case of left atrial spindle cell sarcoma diagnosed and treated by a multidisciplinary approach, and retrospectively reviewed other reported cases.Patient concerns:A 49-year-old woman presented to our hospital with 2 weeks of gradual onset of dyspnea on exertion, dry cough and subacute fever.Diagnosis:The patient was initially revealed a left atrium mass by 2-dimensional transthoracic echocardiography. Based on the contrast-enhanced echocardiography and cardiac magnetic resonance imaging, she was subsequently suggested to have a cardiac malignant tumor. And the post-operative histopathology confirmed the tumor to be a cardiac primary spindle cell sarcoma.Interventions:The tumor was completely resected using autotransplantation. The patient was referred for polychemotherapy afterwards.Outcomes:Our patient underwent the tumor resection, with subsequent adjuvant polychemotherapy, and the tumor has not recurred during 12 months of follow-up.Lessons:Due to the rarity of these tumors and nonspecific symptoms, they are often difficult to diagnose preoperatively and missed occasionally. Thus, improving our understanding of the disease and facilitating its early diagnosis are essential.     

Retroperitoneal dendritic cell sarcoma: A case report

Ratioanle:Interdigitating dendritic cell sarcoma (IDCS) is a rare sarcoma that originates from interdigitating dendritic cells in lymphoid tissue, the imaging characteristics of which are poorly defined. Pathological examination can identify the tumor, but reports on the imaging characteristics of IDCS are limited.Patient concerns:Here, we report a case of IDCS in a 48-year-old female involving the retroperitoneal area. The patient had a lumbar mass on her right lower back for 4 years, and which started increasing in size 1 year before.Diagnoses:An irregular soft tissue mass (10.1cm × 8.5 cm in size) in the right lower back of retroperitoneum was detected by CT examination with unclear borders, uneven density, and necrosis. The solid components of the mass were significantly enhanced on postcontrast imaging. The soft tissue was irregular and uneven. Cystic solid masses were observed on MRI examination in the right retroperitoneum, lateral abdominal wall, waist, and back. Necrosis, hemorrhage, and cystic transformation were observed inside the lesion. The cyst wall, separation, and wall nodules were significantly enhanced on the postcontrast image. No distant metastasis was observed. Postoperative pathology confirmed the diagnosis of IDCS.Interventions:The patient underwent surgical resection. The resected margin was positive, and the patient received adjuvant radiotherapy 2 months after the surgery.Outcomes:Twelve months after radiotherapy, the patient''s chest CT showed multiple metastases in both lungs. The patient was started on combination chemotherapy of doxorubicin and ifosfamide, and the follow-up is still ongoing.Lessons:Imaging provides a unique advantage to determine the extent of the IDCS, the invasion of adjacent tissues, and the presence or absence of distant metastases.     

Heterotopic pancreas in the stomach： A case report and literature review

Ectopic pancreas is defined as pancreatic tissue found outside the usual anatomic location of the pancreas. It is often an incidental finding and can be found at different sites in the gastrointestinal tract. It may become clinically evident when complicated by pathologic changes such as inflammation, bleeding, obstruction, and malignant transformation. In this report, a 40 years old woman with epigastric pain due to ectopic pancreatic tissue in the stomach is described. The difficulty of making an ac- curate diagnosis is highlighted. The patient has remained free of symptoms since she underwent wedge resection of the lesion three years ago. Frozen sections may help in deciding the extent of resection intraoperatively. Although ectopic pancreas is rare, it should be considered in the differential diagnosis of a submucosal gastric tumour.