Case of a huge recurrent retroperitoneal liposarcoma diagnosed in the second trimester of pregnancy

We document a case of a 24-year-old gravida 2, para 1 (1001), on her second trimester, who was referred to the surgical oncology service for a palpable right hemiabdominal mass. She previously underwent wide excision of a retroperitoneal atypical lipomatous tumour 2 years ago. Her current case was successfully managed by a multidisciplinary team of doctors from the preoperative phase, the actual surgery up to the adjuvant treatment. She had a two-staged surgery: an elective lower-segment caesarean section at 34 weeks age of gestation for which she delivered a healthy baby girl and, 2 weeks after, she had excision of the huge retroperitoneal tumour recurrence. Histopathology revealed a 35 cm×25 cm×22 cm dedifferentiated liposarcoma, for which she was started on adjuvant systemic chemotherapy.

Paraneoplastic pemphigus caused by pre-existing stroma-rich variant of Castleman disease: from a pathologist’s point of view

A young woman presented with mucocutaneous blisters and ulcerating lesions, and was diagnosed with erythrodermic pemphigus complicated by bronchiolitis obliterans. Her clinical condition did not improve on immunosuppressive therapy. She had a history of an asymptomatic retroperitoneal mass, presumed to be a dermoid cyst, followed up clinically. Due to the pre-existing nature of the retroperitoneal mass, the paraneoplastic nature of the pemphigus was initially not recognised, but after a multidisciplinary team meeting a biopsy was performed. Histology revealed a rare stroma-rich variant of Castleman disease with a prominent stroma demonstrating a myoid phenotype. Resection of the retroperitoneal tumour resulted in resolution of the cutaneous blisters. This emphasises the importance to consider paraneoplastic disease in treatment-resistant pemphigus as surgical removal of the tumour forms the mainstay of therapy. The differential diagnosis should include Castleman disease and careful evaluation of histology is essential with the awareness of this rare stroma-rich variant.

Haemorrhagic retroperitoneal paraganglioma initially manifesting as acute abdomen: a rare case report and literature review

Background Paragangliomas (PGLs) are extremely rare neuroendocrine tumours arising from extra-adrenal chromaffin cells. PGLs are clinically rare, difficult to diagnose and usually require surgical intervention. PGLs mostly present catecholamine-related symptoms. We report a case of Acute abdomen as the initial manifestation of haemorrhagic retroperitoneal PGL. There has been only one similar case reported in literature. Case presentation We present a unique case of a 52-year-old female with acute abdomen induced by haemorrhagic retroperitoneal PGL. The patient had a 5-h history of sudden onset of serve right lower quadrant abdominal pain radiating to the right flank and right lumbar region. Patient had classic symptoms of acute abdomen. Abdominal ultrasound revealed a large abdominal mass with a clear boundary. A Computed Tomography Angiography (CTA) of superior mesenteric artery was also performed to in the emergency department. The CTA demonstrated a large retroperitoneal mass measured 9.0 × 7.3 cm with higher density inside. A provisional diagnosis of retroperitoneal tumour with haemorrhage was made. The patient received intravenous fluids, broad-spectrum antibiotics and somatostatin. On the 3rd day of admission, her abdominal pain was slightly relieved, but haemoglobin decreased from 10.9 to 9.4 g/dL in 12 h suggesting that there might be active bleeding in the abdominal cavity. Thus, we performed a midline laparotomy for the patient. Haemorrhage was successfully stopped during operation. The retroperitoneal tumour with haemorrhage was completely removed. The abdominal pain was significantly relieved after surgery. The patient initially presented with acute abdomen instead of catecholamine-related symptoms. The diagnosis of retroperitoneal PGL with haemorrhage was finally confirmed by postoperative pathological and immunohistochemical results. The postoperative course was uneventful. At the 1-year follow-up visit, no tumour recurrence was observed by Single Photon Emission Computed Tomography. A literature review was performed to further understand and analyse the aforementioned disease. Conclusion Acute abdomen as the initial manifestation of haemorrhagic retroperitoneal paraganglioma is extremely rare. Abdominal Computed Tomography is essential to locate the lesion and differentiate between other causes of acute abdomen. PGLs are hypervascular tumours. We should be aware that ruptured retroperitoneal PGL with massive bleeding could be life threatening and require emergency laparotomy.

RETROPERITONEAL HEMANGIOMA: A VERY RARE CASE PRESENTATION

Retroperitoneal tumour which can be benign or malignant is a rare entity (of which vascular neoplasms are extremely rare) with late clinical presentation, most often diagnosed in advanced stage of the disease. The complex anatomical location in the retroperitoneum, its inaccessibility and close relation to important structures in the retroperitoneal space pose a greater challenge in the diagnosis and the management of the disease. Here we are presenting a case of retroperitoneal tumour which presented with lump in abdomen since 10 years. Biopsy of the lump was suggestive of benign lesion. Complete surgical excision of the tumour was done and on final histopathology was suggestive of benign vascular neoplasm consistent with venous/cavernous hemangioma. Regular follow-up is uneventful.

RETROPERITONEAL BENIGN FIBROUS HISTIOCYTOMA-A CASE REPORT

Benign fibrous histiocytomas are common soft tissue tumours located anywhere in the body but most commonly found in skin. These are slow growing tumours common in all races and all ages but most common age group is 20 to 49 years with female preponderance. In our case a 27-year-old female with complaints of abdominal pain underwent an ultrasound abdomen and pelvis in which a retroperitoneal tumour was diagnosed. Except for abdominal pain no other complaints were noted she was well preserved. we have done an exploratory laparotomy with excision of tumour and specimen sent for Histopathological examination in which it is confirmed as benign fibrous histiocytoma. Post-operative period uneventful and she recovered well. It is rare site for the occurrence of the histiocytoma.