Unicentric Castleman Disease and Pheochromocytoma

Castleman disease is an uncommon and heterogenous lymphoproliferative disorder which is classified as unicentric or multicentric depending on the number of lymph nodes involved. Each type has a different clinical presentation, aetiology, treatment and prognosis. We report the case of a young woman who presented with cervical lymphadenopathy and a retroperitoneal mass, and was diagnosed with unicentric Castleman disease and pheochromocytoma. We describe the diagnostic steps, the complications that developed, and the importance of the differential diagnosis in the evaluation of these patients.

Retroperitoneal Mass: Lymphoma as Differential Diagnosis to Retroperitoneal Fibrosis: Case Report

The etiology of solid retroperitoneal mass may be autoimmune or neoplastic and should be investigated by imaging and histology. The spectrum of differential diagnoses includes retroperitoneal fibrosis and retroperitoneal tumors. As treatment for these entities differs substantially, early and accurate diagnosis is essential. We present a case of a 54-year-old woman admitted to our hospital with stroke-like symptoms. Suspecting vasculitis, magnetic resonance imaging of the head and abdomen was performed, which revealed circular enhancement of the internal carotid artery as well as retroperitoneal and periaortic masses. In light of the radiographic findings, an autoimmune process, such as retroperitoneal fibrosis, was hypothesized. Steroid treatment was initiated but did not lead to significant remission. Re-evaluation of the mass with fine-needle aspiration did not show malignant cells while diagnostic surgery and histological assessment revealed neoplastic lymphoproliferation. The final diagnosis was a non-Hodgkin B-cell lymphoma. Chemo- and immunotherapy were initiated. Follow-up abdominal computed tomography revealed significant remission of the retroperitoneal mass. Initially, the retroperitoneal mass was highly suspicious for RF. While imaging can be useful, obtaining histology should always be considered when there is an uncertain clinical presentation. Without histology, we would have missed a non-Hodgkin B-cell lymphoma in this case. Minimally invasive techniques such as fine-needle aspiration may be practical but can give false-negative results.

Heterotopic Ossification of the Inferior Vena Cava Wall: A Case Report and Literature Review

Masses of the inferior vena cava (IVC) are very diverse, most of which are thrombus and tumor thrombus, whereas heterotopic ossification of IVC has never been reported. Heterotopic ossification (HO) is the formation of mature lamellar bone outside normal bones and in soft tissues. Some researchers believe that HO is a manifestation of vascular calcification. Here we present a case of HO of the inferior vena cava (IVC) wall. A 68 year old female patient complaining hypertension and palpitation and diagnosed with a retroperitoneal mass was found to have a primary mass of the inferior vena cava wall during surgery. Histopathological examination after surgical resection revealed that the mass was mainly composed of mature bone tissues and hematopoietic tissues of bone marrow, there was no recurrence and the patient was symptom-free 15 months after the surgery. HO of the inferior vena cava wall is very rare, with large volume it can affect the circulation, and this case remind us that it can be cured by surgical resection.

Tumour-Not So Sweet, Tumour-Induced Hypoglycemia: A Rare Case of Refractory Hypoglycemia in a Toddler

Tumour-induced hypoglycaemia is a rare complication/condition mainly seen in adults. It is caused due to increased production of insulin or insulin-like growth factor (IGF) 2 tumour cells. We present a 3-year-old paediatric patient with non-islet cell tumour induced hypoglycaemia (NICTH) secondary to rhabdomyosarcoma. She presented with abdominal mass and refractory hypoglycaemia, requiring high glucose infusion and steroids. Critical sample analysis during hypoglycaemia showed suppression of insulin, IGF-1, C-peptide, growth hormone, and ketones, with a high cortisol level. CT scan of abdomen and pelvis showed a huge retroperitoneal mass, later diagnosed as rhabdomyosarcoma. In a resource-limited setting, where IGF-2 is not possible, low serum insulin and IGF-1 levels during hypoglycaemia aids in diagnosis of NICTH. This is one of the first few reported paediatric cases with NICTH from India, and we believe that reporting this case would add more information to the existing literature. Thus, NICTH should be suspected in all malignancies presenting with intractable hypoglycaemia irrespective of their age.

Retroperitoneal Angiomyolipoma in A Woman of Childbearing Age: A Case Report

Introduction: Extrarenal angiomyolipoma is an uncommon disease entity and is rare in the retroperitoneum. This lesion shows three benign components, including mature adipose tissue, thick-walled blood vessels, and smooth muscles. Case Report: This report describes a 37-year-old woman who presented with incidental right retroperitoneal mass in prenatal checkup. The patient underwent excisional surgery. Histological examinations and the immunohistochemical study revealed angiomyolipoma. Based on the diagnosis, the patient received no more treatment. After 3 years of close followup examinations, no recurrence was observed. Conclusion: This diagnosis is often confused with many other entities in retroperitoneum. Thus, imaging and histologic correlation are required. Proper diagnosis is essential for further patient management and avoids unnecessary treatment.

Imaging findings in Fetus-in-fetu-misdiagnosed commonly as teratoma

Fetus in fetu is a rare condition associated with abnormal embryogenesis in a diamniotic, monochorionic pregnancy, wherein one of the fetus is enclosed within the body of another normally developing fetus. It should be differentiated from a teratoma because of the later’s malignant potential. Here we report a case of 2 months old girl child who presented with complaints of abdominal distension. USG showed a solid cystic retroperitoneal mass resembling an anencephalic fetus. Contrast enhanced Computed tomography (CT) showed similar findings with visualization of bones resembling femur, sacrum and vertebrae. Findings were correlated with MRI and post-op pathology. The preoperative diagnosis of FIF is based on the observation of vertebral column or limbs in a mass on imaging modalities and our case meets the required criteria.

Case Report About Extraperitoneal Metastasis of Cervical Adenocarcinoma In Situ

Background: adenocarcinoma in situ(AIS) cells are often misdiagnosed, and recognizing AIS in cervical cytology is challenging. Here, we present a case of extraperitoneal metastasis 5 years after a missed diagnosis of AIS.Case presentation: We report a 49-year-old Chinese woman who presented with a retroperitoneal mass 5 years after cervical conization for AIS. The retroperitoneal mass was composed of glands lined by malignant mucinous epithelium; these tumors were metastases from her previous AIS which were misdiagonised cervical intraepithelial neoplasia(CIN) III in 2013. The patient is alive and well 2 years after resection of the tumors.Conclusions: An experienced pathologist or multiple pathologists should take part in endocervical AIS diagnosis. We should follow these patients for over 15 years. When Cancer Antigen 125(CA125) or Carcinoembryonic antigen(CEA) levels are elevated, the occurrence of metastases should be strictly monitored.