Localized Castleman's disease in the breast

INTRODUCTIONCastleman's disease (CD) is a rare disease with unknown etiology and is clinically associated with lymph nodes enlargement. Primary axillary localization of CD represents 2% of the cases. CD rarely occurs in the breasts.PRESENTATION OF CASEWe herein describe a rare case of CD that initially presented in the breast intramammary lymph node and demonstrated axillary adenopathy. Pathologic analysis showed the hyaline vascular form. The patient underwent axillary lymphadenectomy. The natural history was irregular because the localized CD progressed to a systemic form of CD. At 4.6 years of follow-up a Hodgkin's lymphoma appeared.DISCUSSIONThis is the fourth published case of localized breast CD published. It is important to evaluate other clinical lymphadenopathies at the time of diagnosis, and computed tomography is important for disease evaluation and follow-up.CONCLUSIONPatients must undergo axillary lymphadenectomy when showing clinical symptoms. Irregular progression prompts new lymph node biopsy because of the potential presence of associated diseases.     

Castleman’s Disease—A Rare Diagnosis in the Retroperitoneum

Abdominal masses have always been a dilemma for the surgeons and this problem is more so in retroperitoneal masses. In our case, the patient presented with retroperitoneal mass in the left lumbar region with no other specific local or systemic symptoms. The diagnosis of Castleman’s disease was established only after histopathological examination of the mass after resection. Castleman’s disease is an angiofollicular lymph node hyperplasia presenting either as a localized or a systemic disease. In our case, the patient presented with the localized form of the disease and as it is a rare presentation we are presenting this case.     

A case of incidentally detected Castleman's disease with retroperitoneal paravertebral localization.

Castleman's disease, or angiofollicular lymph node hyperplasia, is a fairly rare benign tumor of lymphoid origin. The expected localization is mediastinum and rarely retroperitoneum. An asymptomatic case is reported with multimodality imaging and postoperative findings. The lesion was detected incidentally on routine chest radiogram. Surgical exploration revealed a retroperitoneal mass and the mass was resected successfully through a thoracoabdominal flank incision. Castleman's disease should be included in the list of differential diagnosis of retroperitoneal masses which are mostly malignant tumors.     

Castleman's disease in the pelvic retroperitoneum: A case report and review of the Japanese literature

INTRODUCTIONCastleman's disease is a fairly rare benign tumor of lymphoid origin. It can develop anywhere lymphoid tissue is found, but the expected origin is mediastinum and rarely pelvic retroperitoneum.PRESENTATION OF CASEA 22-year-old woman was admitted to our hospital for a mass in the pelvic retroperitoneum that was detected incidentally on an ultrasonography during a routine medical checkup with no signs of symptoms. After laboratory examination, ultrasonography, and magnetic resonance imaging (MRI), surgical resection was performed successfully through a lower midline incision. But the patient was needed transfusion because of massive bleeding. Postoperative histopathological diagnosis was hyaline-vascular type of Castleman's disease. The patient is leading an active social life without any signs of sequelae or recurrence.DISCUSSIONThrough the review of Japanese literature on Castleman's disease in the retroperitoneum, the characteristics of preoperative imaging findings are studied. Castleman's disease is easily misdiagnosed clinically because of its scarcity and no specific imaging findings. And the embolization via angiography should be considered in the hypervasular tumors such as in this case to prevent massive bleeding and transfusion.CONCLUSIONAlthough Castleman's disease is uncommon, it should always be included in the differential diagnosis of pelvic retroperitoneal tumors. A better knowledge of this disease would help surgeon to avoid unnecessarily extensive resection and massive bleeding for transfusion when dealing with retroperitoneal tumors.     

Castleman's disease: an unusual retroperitoneal mass

Castleman's disease (angiofollicular lymph node hyperplasia) rarely presents as an isolated retroperitoneal mass radiographically indistinguishable from retroperitoneal malignancies. We report a case of the hyaline vascular variant of Castleman's disease presenting as a calcified pararenal mass. Computerized tomography and nuclear magnetic resonance imaging of this lesion are presented, and its clinical behavior and management are discussed.     

Rapidly progressive renal failure associated with angiofollicular lymph node hyperplasia.

The authors report a case of rapidly progressive renal failure associated with Castleman's disease. Renal biopsy revealed crescentic glomerulonephritis associated with marked tubulointerstitial nephritis. This kind of renal lesion has never been reported in association with angiofollicular lymph node hyperplasia (Castleman's disease) or other immunoblastic disorders. The patient initially required hemodialysis therapy. However, steroid therapy was effective in treating the systemic manifestations of Castleman's disease and some renal function was recovered. The patient was finally withdrawn from hemodialysis therapy.     

Membranoproliferative glomerulonephritis associated with multicentric angiofollicular lymph node hyperplasia. Case report and review of the literature.

A 14-year-old boy presented with fever, anemia, hepatosplenomegaly, generalized lymphadenopathy and nephrotic syndrome. Lymph node biopsy showed angiofollicular lymph node hyperplasia (generalized Castleman's disease) of the plasma cell type. Kidney biopsy showed membranoproliferative glomerulonephritis type 1. Complete remission was achieved with corticosteroid treatment and repeat kidney biopsy 22 months later showed complete resolution of the renal pathology. The association between membranoproliferative glomerulonephritis and multicentric angiofollicular lymph node hyperplasia, plasma cell type, has not previously been reported.     

Laparoscopic treatment of unicentric Castleman's disease with abdominal localization

We report a case of unicentric Castleman's disease (angiofollicular lymph node hyperplasia) with abdominal localization, that was treated laparoscopically. The patient, a 23-year-old male, was referred to our unit for subtle symptoms of recurrent palpitations and vague abdominal pain. His physician had prescribed an abdominal echtomograph, which showed a mass located at the lower and anterior lower splenic pole. In order to reach a definite diagnosis and prescribe adequate treatment, a diagnostic laparoscopy was performed. Exploration of the abdominal cavity helped detect a well-vascularized solid round mass at the level of the left hypochondrium, with a vascular pedicle; the lesion was detached, and the pedicle sectioned using an Endo-GIA 40. The postoperative course was regular and the patient was discharged on postoperative day 2. The laparoscopic approach enabled the resection of the lesion (with consequent histological diagnosis) and exploration of the peritoneal cavity with the advantages of minimal invasiveness, magnified images, and more rapid recovery). The pathology was totally resolved, with satisfactory results in terms of recovery, postoperative pain, and cosmesis.     

Paraneoplastic pemphigus associated with a pericardial ectopic thymoma

Paraneoplastic Pemphigus (PNP), a rare autoimmune blistering disease, can be accompanied by both benign and malignant neoplasms. The most frequently reported associated malignancies include lymphomatoid and hematologic malignancies, Castleman's disease, carcinoma, thymoma. In a patient suspected of PNP, with no known history of malignancy, an extensive workup is suggested to look for underlying malignancy, which has to be treated to induce PNP remission. In this clinical case report, cross sectional imaging of a young female diagnosed with PNP, unveiled a pericardial mass lesion extending into transverse pericardial sinus. Excisional biopsy was performed. Histopathology revealed pericardial ectopic thymoma.     

Retroperitoneal unicentric Castleman’s disease: A case report

IntroductionCastleman’s disease (CD) is an angio-follicular lymph node hyperplasia presenting as a localized or a systemic disease masquerading malignancy. The most common sites of CD are mediastinum, neck, axilla and pelvis. Unicentric CD in the peripancreatic region is very rare.Presentation of caseWe report a case of the 34-year-old lady presenting with epigastric pain for 3 months. Abdominal imaging revealed a retroperitoneal mass arising from the pancreas suspected to be neuroendocrine tumor. Tumor markers were not elevated. Complete surgical excision was performed and patient had uneventful recovery. Pathologic findings demonstrated localized hyaline-vascular type of Castleman’s disease.DiscussionCD is a very rare cause for development of retroperitoneal mass. It is more frequent in young adults without predilection of sex. It can occur anywhere along the lymphoid chain. Abdominal and retroperitoneal locations usually present with symptoms due to the mass effect on adjacent organs. CD appears as a homogeneously hypoechoic mass on ultrasound and non-specific enhancing homogeneous mass with micro calcifications on computed tomography. Histologically, the hyaline vascular type demonstrates a follicular and inter-follicular capillary proliferation with peri-vascular hyalinization, with expansion of the mantle zones by a mixed inflammatory infiltrate of numerous small lymphocytes and plasma cells. The standard therapy of localized form is en bloc surgical excision as performed in our case.ConclusionUnicentric CD in the peripancreatic region is difficult to differentiate from pancreatic neoplasm preoperatively. However, preoperative biopsy in cases of high clinical suspicion can help in avoiding extensive surgery for this benign disease.     

Extranodal lymphoma of the tongue,a very rare entity-report of two cases with literature review

BackgroundLymphomas are malignant neoplasms of the lymphocyte cell lines affecting the lymph nodes, spleen and other nonhemopoietic tissues. Of the extranodal lymphomas found in the head and neck region, 3–5% of malignant lymphomas arise in the oral and paraoral region, mainly from Waldeyer's ring. The involvement of the base of the tongue is extremely rare.Summarised CaseCase 1: 64 year old female who presented initially with an enlarged occipital lymph node which gradually became generalized cervical lymphadenopathy with initial histology confirmed reactive lymphoid hyperplasia. Biopsy of left postero-lateral tongue lesion eventually showed high grade T cell lymphoma.Case 2: 85 year old male presented with history of dysphagia for one year who was found to have a lesion extending from his base of tongue into the nasopharynx. Histology showed a diffuse B cell lymphoma.DiscussionBoth patients were noted to have lesion of the tongue, but tongue lesions are noted in the literature to be extremely rare. When tongue lymphomas do occur, most are of B-cell origin; the diffuse large-cell variety is the most common. Extranodal lymphomas of the T cell phenotype tend more to be sinonasal in origin than of the tongue, with T cell lymphomas of the tongue being even rarer than B cell lymphomas.ConclusionWith regards to tumours arising in the tongue, squamous cell carcinomas are still classified as the most common. Lymphomas however, should still be kept in consideration as a differential diagnosis with regards to lesions arising from this site.     

Pseudo-adrenal incidentaloma: magnetic resonance imaging in a patient with para-adrenal Castleman's disease.

We report a case of para-adrenal angiofollicular lymph node hyperplasia (Castleman's disease) of the hyaline-vascular type. The mass could not be differentiated from an adrenal tumor by ultrasonography and computed axial tomography (CT). However, magnetic resonance imaging (MRI) suggested the possibility of an extra-adrenal origin of the mass. The intensity of the mass by MRI was homogeneous and of a higher intensity in the T2-weighted image than in the T1-weighted image, a finding similar to lymphadenopathy, lymphatic tumorous mass or metastatic tumor of the lymph node. Ultrasonography, CT and MRI may not be useful in characterizing Castleman's disease, but MRI was useful to distinguish asymptomatic para-adrenal masses from those of adrenal origin.     

Intracranial localized Castleman's disease. Case report

A 68-year-old woman presented with generalized clonic seizure following a 2-month history of initiative loss, incoherent speech, headache, and left hemiparesis. No systemic signs or symptoms were seen and laboratory studies were within normal range. Computed tomography and magnetic resonance imaging demonstrated a well-delineated small mass with homogeneous enhancement in the right parietal convexity, associated with unusually extensive perifocal edema compared to the size of the mass. Cerebral angiography showed a faint stain fed by the middle meningeal artery. These imaging features were very similar to those of meningioma. Full recovery from the symptoms was achieved by total removal of the lesion and no recurrence was found after 3 years. Histological examination identified the hyaline-vascular type of angiofollicular lymph node hyperplasia (Castleman's disease). Castleman's disease involving the central nervous system is rare, with only 12 previous cases, but should be considered in the diagnosis of intracranial meningeal tumors. The treatment of choice for localized Castleman's disease is complete surgical resection, which is curative in most of the cases.     

Metachronous common iliac lymph node metastasis after rectosigmoid colon cancer resection: A case report

Introduction and importanceMetastases to common iliac lymph nodes from cancer of the rectosigmoid are extremely rare. We report a patient with a right common iliac lymph node metastasis after rectosigmoid cancer resection.Case presentationThe patient is a 57-year-old woman diagnosed with rectosigmoid cancer (Stage IIIc) who underwent laparoscopic resection followed by 8 courses of adjuvant chemotherapy with capecitabine. Sixteen months after resection, an intra-abdominal mass and a left lung nodule were found on computed tomography scans, which were suspected to be recurrences. Exploratory laparoscopy showed that the abdominal lesion was an enlarged common iliac lymph node, which was completely excised. No other intraabdominal recurrences were found. Subsequently, a left upper lobe lung metastasis was resected thoracoscopically. However, multiple lung metastases developed four months after the lung resection, and systemic therapy was begun.Clinical discussionA lower incidence of lateral lymph node metastases from cancer in the rectosigmoid has been reported. Direct lymphatic pathways from the sigmoid colon or rectosigmoid to lateral lymph nodes have been suspected, which may be associated with the poor prognosis in this patient.ConclusionA metachronous metastasis to a common iliac lymph node from primary rectosigmoid cancer is reported. Common iliac lymph node metastases from rectosigmoid cancer might have more malignant potential, and should be treated in the same manner as peri-aortic lymph node metastases.     

Ischiorectal fossa metastasis from colon cancer: Case report of a rare entity and review of literature

Introduction and importanceColorectal cancer is one of the most common cancers both nationally and internationally. It commonly metastases to local lymph nodes, liver and lungs, with few reported cases of rare sites of metastasis such as adrenal glands, breast and skin.Case presentationWe report a 55-year-old-female admitted as case of large bowel obstruction and unintentional weight loss. Computed tomography scan of chest, abdomen and pelvis (CT CAP) showed sigmoid colon circumferential thickening with three lesions in the right hemi-liver. A laparoscopic diverting ileostomy followed by a colonoscopy showed a sigmoidal mass consistent with adenocarcinoma on histopathology. Hence, she received neoadjuvant chemotherapy followed by hepatectomy for the liver metastasis. Post-operatively CT CAP showed a newly developed right ischiorectal fossa (IRF) nodule along with newly developed porta hepatis lymph node. PET scan showed uptake in these two new lesions. Therefore, the patient underwent resection of the primary tumor, porta hepatis lymph node and right ischiorectal fossa nodule excision. The histopathology of the primary tumor came as moderately differentiated adenocarcinoma with both ischiorectal lesion and the porta hepatis nodule being positive for metastatic disease.Clinical discussion & conclusionIschiorectal fossa tumors are extremely rare with the majority being benign in origin. Nevertheless, the possibility of metastasis is there with no clear explanation regarding the pathway of how the metastatic cells can reach the IRF. Pre-operative diagnosis is important to determine the appropriate approach particularly if the mass is thought to be malignant. Further larger studies are needed to understand the pathway of metastasis to IRF.     

Castleman's disease in two patients with spondyloarthritis treated by Tumor Necrosis Factor-alpha antagonists

Castleman's disease is a rare lymphoproliferative syndrome involving several anatomical and clinical entities. We report two cases of Castleman's disease in patients with spondyloarthritis (with and without psoriasis) treated with Tumor Necrosis Factor-alpha blockers. During follow-up, imaging examinations, carried out for disease assessment, fortuitously revealed a pelvic mass close to the psoas or an atypical cervical adenopathy. Both lesions were in moderate hypermetabolism on Positron emission tomography–computed tomography. After surgical excision and histological analysis, Castleman's disease was diagnosed without signs of malignancy. To the best of our knowledge, these are the first cases of Castleman's disease diagnosed in a context of spondyloarthritis treated with Tumor Necrosis Factor-alpha blockers. The relationship between these two pathologies and the link with Tumor Necrosis Factor-alpha blockers agents has not been explored in the literature until now.     

Retroperitoneal unicentric Castleman's disease—A case report and review of literature

Introduction and importanceCastleman's disease is not so commonly diagnosed worldwide due to non-specific symptoms Clinical findings are variable.No definite blood investigation or any biomarkers are established to diagnose this disease. Radiological investigations do not play much role in diagnosing. It can be unicentric or multicentric. Etiological factors are not well understood except predilection of this disease towards immune-compromised persons. Surgery is considered as a prime modality to treat, if resectable.Case presentationPatient had recurrent abdominal pain in left lumbar region which was intermittent for last 6 months, dull aching with no aggravating and relieving factors. There was no history of abnormal bowel habits, urinary complaints, fever, night sweats, weight loss, rashes in body, joint pains and loss of appetite. On examination, there was a single, firm, non-tender, intra-abdominal, retroperitoneal mass of approximately size 10 1 7 cm in left lumbar region encroaching inferiorly towards left inguinal region. Digital rectal examination (DRE) and external genitalia were normal.Clinical discussionAll routine blood investigations along with relevant tumour markers were normal. Magnetic Resonance Imaging (MRI) abdomen showed a well-defined mass suggestive of stromal tumour or retroperitoneal sarcoma. Patient underwent surgery in which complete excision of the mass was done. Postoperative event was uneventful and currently doing well.ConclusionTake away lesson in this case report is that we should not presume all retroperitoneal mass as cancer or sarcoma, we have to think about other rare causes like Castleman's disease, if clinical picture, blood and radiological investigation are discordant.     

Mesentery lymphoma in a patient with Crohn's disease: An extremely rare entity

INTRODUCTIONLymphoma is a rare complication of long-standing Crohn's disease. We report a rare case of a diffuse, B-cell non-Hodgkin's lymphoma of the mesentery in a patient receiving treatment for Crohn's disease.PRESENTATION OF CASEA 52 year-old patient presented with abdominal pain, anorexia and postprandial fullness. Abdominal examination revealed a firm mass, extending from the epigastrium to the right iliac fossa. CT scan showed a large intra-abdominal mass with air-fluid levels within, and soft tissue density along its walls, surrounded by distended bowel loops. The patient was scheduled for surgery due to clinical assumption of an intra-abdominal abscess. At laparotomy an ill-defined, lobulated mass with cystic areas was noted rising from the mesentery. Frozen section biopsy of the cystic mass revealed a non-Hodgkin follicle center B-cell lymphoma of the mesentery.DISCUSSIONTo the best of our knowledge, this is an extremely rare case of lymphoma development in the mesentery, in a patient receiving treatment for Crohn's disease. Although the development of abdominal lymphomas can be justified as a possible consequence of the chronic immune-modulating therapy, their location can lead to diagnostic pitfalls.CONCLUSIONAlthough mesentery has scarcely been presented as a potential site of occurrence of abdominal lymphomas in the process of treatment of inflammatory bowel diseases, this rare entity should be considered in the differential diagnosis of intra-abdominal lymphomas in patients with inflammatory bowel disease. In cases where imaging techniques do not provide definitive answers, surgical intervention can safely pose the accurate diagnosis.     

An exceptional cause of renal colic. Castleman's tumor. Lymphonodular angiofollicular hyperplasia

The case of a 38-year-old man with renal colic due to compression by an abdominopelvic mass is reported. Histologic examination of the mass led to the diagnosis of giant lymph node hyperplasia or Castleman disease. Surgical removal of the lesion ensured complete recovery with no recurrence after 32 months follow up. Giant lymph node hyperplasia usually develops in the mediastinum, but superficial and abdominopelvic forms are seen occasionally. Pathogenic hypotheses are discussed. Outcome is usually favorable following surgical excision but rare multifocal forms with a potentially poor prognosis have been reported.