Left Retroperitoneal Lymph Node Research Articles

Seminoma arising in splenogonadal fusion: a case report and literature review

BackgroundSplenogonadal fusion (SGF) is a rare congenital malformation in which the spleen is abnormally connected to the gonads or to the mesonephric derivatives. There is no obvious causality between SGF and testicular neoplasm. However, cryptorchidism, which is a well-known risk factor of testicular germ cell tumors, are the most frequent malformations associated with SGF. To our knowledge, there are only four reported cases of SGF associated with testicular neoplasm so far. Herein, we reported a patient of this condition, and briefly reviewed the related literature.Case presentationA 48-year-old man was diagnosed with bilateral cryptorchidism 30 years prior, and only underwent a right orchiopexy for the left testicle could not be explored during the operation. At that time, doctors failed to realize the possibility of SGF due to the lack of sufficient knowledge of this condition. This time, the patient was treated for a left abdomen mass that was diagnosed as stage III metastatic seminoma. Then, a right orchiectomy, robot-assisted laparoscopic left retroperitoneal tumor resection, and left retroperitoneal lymph node dissection was performed after four cycles of BEP (bleomycin + etoposide + cisplatin) systemic chemotherapy in our center. The final diagnosis of SGF was made by postoperative pathology. The patient was re-examined in our center at 3 months and 6 months after the operation, and no obvious abnormalities were found.ConclusionsSurgeons should always bear in mind the possibility of association between bilateral cryptorchidism and splenogonadal fusion to avoid malignant transformation caused by delayed treatment.

Intensity modulated radiotherapy and targeted therapy for late nonregional para-aortic lymph node metastasis from HER2 positive breast carcinoma

In the case of invasive breast carcinomas (BC) there are extremely rare late nonregional retroperitoneal lymph node recurrences, occurring after 10 years of disease diagnosis. We present a 32-year-old woman, who was diagnosed in 2010 with left HER2 positive invasive ductal BC- pT2N1M0. Complex treatment involving radical mastectomy with axillary dissection and complex adjuvant treatment (chemotherapy, radiotherapy, targeted therapy with trastuzumab, endocrine therapy with LHRH agonist plus tamoxifen) was conducted. After 4 years, disease progression with left supraclavicular lymph node enlargement has been manifested. After surgical resection of supraclavicular lymph node, the patohistological analysis establishes lymph metastasis from HER2 positive invasive ductal carcinoma. After 6 years of multimodal treatment, including eight chemotherapy cycles docetaxel, bilateral adnexectomy, 2 targeted agents trastuzumab/pertuzumab and endocrine therapy with aromatase inhibitor, PET/ CT visualizes a nonregional lymph recurrence of left retroperitoneal lymph nodes. Isolated involvement of distant nodal regions is extremely uncommon. Complex therapy, including a definitive radiotherapy of retroperitoneal para-aortic lymph nodes combined with targeted therapy achieved complete remission in nonregional abdominal lymph recurrence.

Abscopal effect of radiation on lymph node metastasis in esophageal carcinoma: A case report and literature review.

The Abscopal effect is a rare phenomenon observed in the treatment of metastatic cancer, where localized irradiation causes a response in non-irradiated tumor sites. Due to the recent success of immunotherapies, the Abscopal effect of radiation therapy has received renewed clinical interest. However, there is limited knowledge regarding the Abscopal effect and radiotherapy treatment of patients with esophageal carcinoma. The present study reports the case of a 65-year-old male patient, who presented with esophageal carcinoma and lymph node metastasis. A transthoracic esophagectomy with left cervical, mediastinal and abdominal lymphadenectomies was performed. A total of 4 cycles of chemotherapy and maintenance therapy with Pembrolizumab was performed until September 2016. Metastases in the left retroperitoneal lymph node in addition to extensive metastases to the pelvic lymph node were observed. The patient received Cyberknife radiotherapy with a dose of 42 Gy in 6 daily fractions targeted at the left retroperitoneal lymph node. Two months after radiation therapy, a positron emission tomography-computed tomography scan revealed complete regression of all lymph node metastases. There is increasing clinical evidence supporting the efficacy of the Abscopal effect, which may be initiated by high-dose radiation. Further research is required to make the Abscopal effect clinically relevant, however it may have potential as a treatment option.

180 Sequential Development of Diffuse Large B-Cell Lymphoma in a Patient With Peripheral T-Cell Lymphoma: Challenges and Risk Factors

We report an extremely rare case of diffuse large B-cell lymphoma sequentially arising in a 61-year-old male patient with CD30-positive peripheral T-cell lymphoma, 9 months after his initial presentation. The patient presented to hospital with extensive pelvic retroperitoneal and inguinal lymphadenopathy. Histologic examination of the left inguinal lymph node excisional biopsy was interpreted as peripheral T-cell lymphoma, not otherwise specified, CD30 positive, Epstein-Barr virus (EBV) positive. The tumor cells were positive for CD3, CD4, CD5, CD30, CD20 (subset), and BCL6 (small subset), with partial loss of CD2 and near complete loss of CD7 and BCL2 by IHC. EBV encoding region in situ hybridization was positive in 10%-20% of lymphoma cells. Flow cytometry showed an abnormal T cell population with polyclonal B cell population. PET CT was positive for bone involvement. Cytogenetics showed a normal male karyotype, and no evidence of clonal T- or B-cell gene rearrangement was detected by PCR. The patient received six cycles of CHOEP (cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone) chemotherapy. Post chemotherapy, PET-CT revealed good response but with residual disease warranting a second biopsy from the left retroperitoneal lymph node, this time showing diffuse large B-cell lymphoma, germinal center type, CD30 negative, EBV negative. The tumor cells were positive for CD10, CD20, and PAX5, and negative for CD2, CD3, CD30, ALK1, and Cyclin D1. They were kappa restricted by flow cytometry. BCL2 gene rearrangement was detected by FISH. He was started on R-GDP (rituximab, gemcitabine, dexamethasone, cisplatin) and completed six cycles but never achieved complete remission. Immunohistochemistry, flow cytometry, molecular studies, and cytogenetics performed during the course of disease confirmed two clonally distinct T- and B-cell lymphomas. We discuss here this unique and challenging case along with the possible etiology and risk factors involved in its development.

A Rare Variant of High-Grade Neuroendocrine Tumor in a Healthy African American Male

Neuroendocrine tumors (NET) are a set of rare tumors composed of glandular endocrine cells typically found in the pancreas, intestine, or lungs. However, the incidence of NET is growing, from 2.48 to 2.586 per 100,000 per year. This growing prevalence stresses the importance in making NET a part of the differential for gastrointestinal (GI) symptoms. Neuroendocrine cells comprise of glandular endocrine cells that reside in the epithelial lining of the GI tract. Classified as either high grade or low grade, NETs are a disruption of these cells. In this case, we present a male with a rare variant of high grade NET. A 51 year old African American male with a past medical history of hypertension and benign prostatic hyperplasia presents to Larkin Community Hospital with abdominal pain for 6 weeks. He complained of constipation for one month with melena, hematochezia, nausea, and abdominal pressure with eating. Abdominal imaging showed multiple heterogeneous retroperitoneal and mesenteric lymph nodes with central hypodensities and rim enhancements, moderate thickening of the gastric antrum, and pylorus. Pathology of left retroperitoneal lymph nodes showed moderately differentiated carcinoma embedded in fibrous tissue. Tumor cells stained positive for CDX2 and negative for CK20, PSA, TTF1. Colonoscopy revealed a friable, ulcerated lesion in the proximal transverse colon. Biopsy showed an infiltrating high grade/large cell NET similar in morphology to the retroperitoneal lymph node biopsy and fragments of tubulovillous adenoma. Transverse colon resection showed extensive lymphovascular invasion, metastatic carcinoma involving 14/14 lymph nodes with extranodal extension and multiple tumor deposits in pericolonic tissue. NETs are defined based on their anatomic location, such as the pancreas, intestine, or lungs. A positive CDX2 suggests a GI origin and TTF1 suggests a pulmonary origin. NETs are comprised of Kulchitsky cells, which help intestinal motility and secretion. Due to the slow growth rate of NET and nonspecific symptoms, diagnosis is delayed until acute bowel obstruction or liver metastases leading to carcinoid syndrome occurs. Two thirds of NETs are carcinoid tumors and one third are of unknown primary origin including high grade/large cell NET as seen in this case. As a result of increasing NET prevalence, it is now imperative to remember to incorporate NETs in the differential diagnosis of atypical masses to prevent delayed diagnosis and treatment.Figure: A friable ulcerated lesion visualized in the proximal transverse colon.Figure: Another view of the friable ulcerated lesion visualized via colonoscopy.

Tumour microemboli as a cause of death in a patient with metastatic urothelial carcinoma

Tumour microemboli are a rare but underdiagnosed complication of end-stage cancer. Diagnosis is challenging and most cases are diagnosed retrospectively at autopsy. Pulmonary tumour microemboli can cause progressive shortness of breath and respiratory failure. They may present in an insidious manner, similar to multiple thromboemboli. This case study reports a patient with a past history of urothelial carcinoma, who presented with increasing dyspnoea and hypoxia. He was found to have pulmonary hypertension, enlarged abdominal lymph nodes and deranged liver function tests. He died suddenly 4 days after admission. At autopsy the patient was found to have tumour microemboli throughout the lungs and liver, in addition to urothelial carcinoma present in the left retroperitoneal lymph nodes, left adrenal gland and bone marrow. Antemortem recognition of tumour microemboli can prevent over-investigation and anticoagulation of patients who may be more appropriate for palliative care.

Smouldering systemic mastocytosis with lymph node involvement mimicking malignant lymphoma

Dear Editor, A 65-year-old male was referred for assessment of enlarged abdominal lymphadenopathy (Fig. 1a) detected by a CT/ abdomen 3 years ago. CT scan also demonstrated that the vertebral bodies and pelvic bones had diffusely heterogeneous appearance which might relate to sclerotic disease (Fig. 1b). A repeated CT/abdomen confirmed a progressive lymphadenopathy in the retroperitoneal and mesenteric region with splenomegaly and sclerotic bone lesions. Patient had no constitutional symptoms. Laboratory investigations including CBC and LDH were normal. A core biopsy on the left retroperitoneal lymph node was nondiagnostic. A bone marrow examination was performed, but due to a dry tap, the aspirate cannot be evaluated. The biopsy revealed abnormal bone trabecular with focal new chaotic bone formation. Trilineage hematopoiesis was preservedwithout lymphocyte aggregates. In variable foci, there were clusters of spindle-shaped cells presented with single elongated/round nuclei along with background eosinophils. Some of these clusters had a perivascular distribution encircling the blood vessels, while others had a parafollicular pattern encircling the hematopoietic tissue. Further, some abnormal cells possessed a paratrabecular pattern, cuffing the bone trabecular (Fig. 1c). The abnormal cells accounted for 30-40 % of bone marrow mononuclear cells, morphologically suggestive of mast cells. The abnormal cells were positive for tryptase, CD25 (Fig. 1d, e), CD117, and CD68, confirming that they were neoplastic mast cells. CD3 and CD20 showed no lymphoma involvement in the bone marrow. According to WHO systemic mastocytosis (SM) diagnostic criteria, it met the major criteria and two minor criteria. Follow-up serum tryptase level was significantly elevated with >200 ng/mL. As the C-kit mutation D816V was equivocal with the peripheral blood samplings and the clinical presentation was atypical, a concurrent low-grade lymphoma could not be ruled out, thus a surgical procedure was performed to have an excisional biopsy on both small bowel mesenteric and retroperitoneal lymph nodes along with a repeated bone marrow examination. The marrow aspirate demonstrated increased abnormal spindle-shaped mastocytes (Fig. 1f), and the biopsy again confirmed SM. Lymph node biopsies showed widely spaced small primary follicles with benign germinal center, the paracortex illustrated diffuse, bland appearing pale spindled cells with reniform vesicular nuclei (Fig. 1h), compatible with neoplastic mast cells, which is highlighted by intensely positive CD117 (Fig. 1g) and CD25 staining. No lymphoma cells were detected, which confirmed the SM involvement in the lymph nodes. C-kit mutation D816V was detected with bone marrow aspirate samplings. Thus, our patient met the major criteria and all four minor criteria. SM is a clonal disorder of neoplastic mast cells which accumulate in human organ systems, especially in bone marrow and skin [1, 2]. Lymph node involvement with significant lymphadenopathy is extremely rare [3]; when involved, it can mimic malignant lymphoma, creating a diagnostic challenge. Z. Xu (*) Division of Hematopathology and Transfusion Medicine, Department of Pathology and Laboratory Medicine, The Ottawa Hospital, General Campus, 501 Smyth Road, Ottawa, Ontario, Canada K1H 8L6 e-mail: zxu@toh.on.ca

Large animal model for retroperitoneal lymphatic and lung metastasis

Retroperitoneal lymph node and lung metastasis are important prognostic factors for gynecologic cancer. The present study aimed to develop a new animal model for retroperitoneal lymph node and lung metastasis. VX2 squamous cell carcinoma tumor tissues were injected into the left gastrocnemius muscle of 38 healthy female New Zealand white rabbits. Animals were randomized into three groups according to day of sacrifice: 1, day 19; 2, day 22; and 3, day 25. Implanted primary tumor (IPTu), left and right retroperitoneal lymph node volumes and lung wet weights were measured on the day of sacrifice. The IPTu and left and right retroperitoneal lymph node volumes increased in a time-dependent manner. In addition, the proportion of animals with metastasis to the left peritoneal lymph nodes and the number of nodes involved increased over time. For days 19, 22 and 25, the proportion of animals with nodal metastasis was 58.3, 84.6 and 100%, respectively, and the number of affected nodes (range) was 3 (2–3), 3 (3–5) and 4 (4–5), respectively. No metastasis was detected in the right peritoneal lymph nodes. Metastasis to the lungs also increased with time, but was not statistically significant at days 19, 22 and 25 with metastasis present in 33.3, 38.5 and 76.9% of animals, respectively. Rates of metastases to the left retroperitoneal lymph nodes and lungs were found to positively correlate with the volumes (r=0.416 and 0.449, respectively). The current study assessed the characterization of a rabbit VX2 carcinoma model. This animal model is likely to be useful for evaluating retroperitoneal lymph node and lung metastasis.

Retroperitoneal teratoma with somatic malignant transformation: A papillary renal cell carcinoma in a testicular germ cell tumour metastasis following platinum-based chemotherapy

BackgroundMalignant transformation describes the phenomenon in which a somatic component of a germ cell teratoma undergoes malignant differentiation. A variety of different types of sarcoma and carcinoma, all non-germ cell, have been described as a result of malignant transformation.Case presentationA 33-year-old man presented with a left testicular mass and elevated tumour markers. Staging investigations revealed retroperitoneal lymphadenopathy with obstruction of the left ureter and distant metastases. Histopathology from the left radical orchiectomy showed a mixed germ cell tumour (Stage III, poor prognosis). The ureter was stented and four cycles of cisplatin, etoposide and bleomycin chemotherapy administered. After initial remission, the patient recurred four years later with a large retroperitoneal mass involving the renal vessels and the left ureter. Left retroperitoneal lymph node dissection with en-bloc resection of the left kidney was performed.Histopathology revealed a germ cell tumour metastasis consisting mainly of mature teratoma. Additionally, within the teratoma a papillary renal cell carcinoma was found. The diagnosis was supported by immunohistochemistry showing positivity for AMACR, CD10 and focal expression of RCC and CK7. There was no radiological or histo-pathological evidence of a primary renal cell cancer.ConclusionsTo the best of our knowledge, malignant transformation into a papillary renal cell carcinoma has not been reported in a testicular germ cell tumour metastasis following platinum-based chemotherapy. This histological diagnosis might have implications for potential future therapies. In the case of disease recurrence, renal cell cancer as origin of the recurrent tumour has to be excluded because renal cell carcinoma metastases would not respond well to the classical germ cell tumour chemotherapy regimens.

A 2-year-old boy with a stage III yolk sac tumor occurring in an intra-abdominal retained testis

A major complication of retained testes is an occurrence of malignancy later in life. We, herein, report the case of a 2-year-old boy who presented with a huge yolk sac tumor with retroperitoneal lymph nodes metastasis that originated in a left intra-abdominal undescended testis. Computed tomography and magnetic resonance imaging showed a huge round tumor connecting to the left retroperitoneal lymph nodes with metastasis extending from the left pelvic region to the left renal hilum. The serum α-fetoprotein level was 36,528 ng/mL. The right abdominal tumor appeared to be a giant testis that had strangulated at the neck of the cord. The tumor had ruptured at the side of the left pelvic lymph node metastasis, and a yolk sac tumor was diagnosed from a histologic analysis of the resected specimens. Postoperative PEB chemotherapy was effective, and a complete surgical resection of the tumor was performed 3 months after the initial laparotomy. The pathologic findings showed fibrous tissue without any tumor cells. The patient has been doing well for 18 months after the radical operation. This case might be a coincidental association of a yolk sac tumor occurring in an undescended testis, which thus caused a delay in making an accurate diagnosis.

I-123 MIBG Scan of Metastatic Neuroblastoma Mimicking a Bone Scan

A 21-year-old man presented to an outside institution with longstanding right hip pain. Magnetic resonance imaging revealed diffuse marrow infiltration in the lumbar spine, sacrum, ilium, and right proximal femur. Biopsy of a left upper quadrant mass was consistent with stroma poor neuroblastoma with differentiation. The patient underwent left radical adrenalectomy, left retroperitoneal lymph node dissection with gross resection of residual tumor and 5 cycles of high-dose chemotherapy (cyclophosphamide, doxorubicin, and vincristine) with evidence of disease progression. A short course of irinotecan was given for palliation. A protocol with low-dose 1-131 MIBG was started for symptomatic control.