Primary omental liposarcoma presenting with torsion: a case report.

An 83 year-old man was admitted because of abdominal pain and distention. A mass measuring 20x10 cm and associated with tenderness and guarding was palpable in the right lower abdomen. Ultrasonography and computed tomography showed a tumor with solid and multiple cystic areas. Celiac angiography showed slight tumor vascularity fed by the right gastroepiploic artery. A malignant tumor of the omentum was suspected. At laparotomy, torsion of the omental pedicle of the tumor was found. Histological examination of the resected tumor revealed characteristics of round-cell liposarcoma, which usually has a poor prognosis. The patient has been alive and well for 2 years. Primary liposarcoma of the omentum has been reported in only seven previous cases. None of these patients presented with torsion, and no report has thus far included a documented survival.     

Retroperitoneal giant angiomyolipoma diagnosed post-partum with lymph node involvement

HISTORY AND PRESENTING COMPLAINT: A 30-year-old primipara after a normal pregnancy had delivered a 3340 g child. After an uneventful post-partum period she had noticed her abdomen failing to reduce in size. INVESTIGATIONS, DIAGNOSIS AND TREATMENT: The abdominal sonography discovered a large retroperitoneal tumor. CT and MRI showed a giant tumor which originated from the right kidney. Suspecting the diagnosis of renal liposarcoma the kidney and tumor were excised with removal of enlarged precaval and preaortal lymph nodes. Gross inspection revealed a ca. 3,2 kg myxoid tumor, measuring 27 x 19 x 10 cm. The histological examination of the surgical preparation revealed a retroperitoneal angiomyolipoma. CONCLUSION: This is the first case of a giant retroperitoneal angiomyolipoma with lymph node involvement diagnosed post partum.     

Complex myxoma detected by syncope: a case report

A 23-year-old man was admitted to our hospital for evaluation of syncope and intracardiac masses. Echocardiography revealed three masses in the right ventricle and one in the left ventricle. The largest mass, 4 by 5 cm, occupied the right ventricular outflow tract and prolapsed through the pulmonary valve orifice. Right ventricular systolic pressure was 65 mmHg. A soft mass, 4 by 5 cm, was found on the left subcostal abdominal wall and multiple pigmented spots on the face and trunk. Histological examination of the resected tumors, including the abdominal soft mass, were consistent with myxoma. The combination of multiple cardiac and abdominal wall myxomas and pigmented skin lesions in this young patient is considered to be a diagnostic feature of Carney complex.     

A case of renin-producing adrenocortical cancer

Here we report a case of a renin-producing adrenocortical carcinoma. A 57-year-old woman was referred to our hospital complaining of thirst and generalized muscle weakness. She was diagnosed as being hypertensive and diabetic with associated hypokalemia and she had a hard elastic mass with a diameter of 10 cm on the left side of her neck. An abdominal computed tomography scan revealed a suprarenal mass on the left side (8.5 x 8 x 6.5 cm). Endocrinological examination demonstrated a marked elevation in the patient's serum glucocorticoid and sex steroid hormones as well as plasma renin activity. Histological examination of a sample taken from the neck mass revealed a metastasis from an adrenal carcinoma, which was stained positively with antibodies against cytochrome P450 and renin, establishing the diagnosis of a renin-producing adrenocortical carcinoma. Trilostane was effective in reducing serum cortisol levels, but mitotane was ineffective.     

A rare case of synchronous adrenocortical carcinoma and renal cell carcinoma

We present here a rare case of synchronous adrenocortical carcinoma (ACC) and renal cell carcinoma (RCC). A 27-year-old woman presented with gradual abdominal distension, hematuria, and loss of weight of 3-months duration. She gave a history of treatment for hypertension. The computed axial tomography (CT) scan revealed a large retroperitoneal mass. Her urinary VMA was slightly elevated. Her 24-h urinary metanephrine level was normal. The patient underwent left adrenalectomy, left nephrectomy, spleenectomy, and distal pancreactomy with segmental colonic resection. Postoperative pathology revealed ACC of left suprarenal measuring 22 × 19 × 18 cm(3) and RCC involving the left upper pole of kidney measuring 3 × 2 × 1 cm(3).     

Hand-assisted laparoscopic radical nephrectomy of renal cell carcinoma--a case report

A 62-year-old female patient with chronic medical history of DM and hypertension was admitted due to traffic accident. She was asymptomatic until 5 days before admission. She was referred to our medical center with an abdominal CT revealing left renal tumor. The diameter of the renal mass was 50 mm. On ultrasonographic examination, a heterogenous lesion over the left kidney was noted. Renal scanning showed a cold nodule in the left kidney. Hand-assisted laparoscopic transperitoneal radical nephrectomy was performed smoothly. The kidney was removed from the hand port in the supraumbilical skin incision with a volume of 14 x 7 x 6 cm3 and weight of 570 gm. The operation time was 5 hours, blood loss was 200 cc, and no significant change of blood pressure was noted during the surgery. The patient's postoperative course was uneventful. The pathological report of this mass was renal cell carcinoma.     

Liposarcoma of the stomach: Report of two cases and review of the literature

Liposarcoma of the stomach is extremely rare, and only 37 cases have been reported worldwide. We herein report two cases of liposarcoma of the stomach. The first patient was referred to our hospital with upper abdominal discomfort. The endoscopic examination revealed a tumor mass about 3 cm in diameter. The patient underwent a partial gastrectomy and had an uneventful recovery. The histopathological examination revealed a welldifferentiated liposarcoma. The second patient had symptoms of upper abdominal discomfort combined with nausea and anorexia. Several palpable masses were found with endoscopy. Endoscopic submucosal dissection was the treatment used, and the postoperative course was uneventful. The histopathological diagnosis was a well-differentiated liposarcoma. The two patients did not undergo any adjuvant therapy. They are both currently in good condition without recurrence. Therefore, we believe that the outcome of liposarcoma of the stomach is positive, and surgical resection may be the first choice for treatment at present.     

Leiomyosarcoma of Renal Vein,Initially Resembling Pheochromocytoma

We report the case of a 43-year-old woman who presented with mild left-sided abdominal pain. Computed tomography and MRI findings revealed a 10.7 × 8.5 × 12 cm left-sided soft tissue mass on the renal vessels pushing away the left kidney. The combination of the patient’s medical history (episodes of paroxysmal hypertension accompanied with dizziness and sweating) and radiological findings initially pointed toward the diagnosis of a paraganglioma or ectopic pheochromocytoma. Surgical removal of the tumor and left nephrectomy was performed. Histological findings were consistent with a renal vein leiomyosarcoma of a high malignancy grade. There is no evidence of local recurrence or metastatic disease for more than 2 years postoperatively, although the patient did not receive adjuvant chemotherapy or radiation therapy.     

Giant extrarenal retroperitoneal angiomyolipoma: a case report and literature review

Extrarenal angiomyolipoma is an uncommon disease entity and is rare in the retroperitoneum. We report a case of giant extrarenal retroperitoneal angiomyolipoma mimicking liposarcoma. A 35-year-old woman presented with a history of increased abdominal circumference and urinary frequency. Abdominal computerized tomography demonstrated a 24 x 21 x 16 cm retroperitoneal fatty tumor displacing the right kidney to the left upper quadrant of the abdomen. At laparotomy, the tumor was widely excised and the right kidney was successfully preserved. Final histologic examination identified the tumor as an angiomyolipoma. At 4-month postoperative follow-up, no evidence of tumor recurrence was found and the right kidney was fully functional.     

Liposarcoma of the stomach： A rare case report

Liposarcoma is the most common soft tissue sarcoma and accounts for 15%-20% of all mesenchymal malignancies. The tumor occurs most frequently in the limbs, retroperitoneum and rarely has a visceral location. We report a case of a gastric liposarcoma in a male patient. A 68 years old male patient was admitted to hospital for abdominal discomfort and fullness lasting for a month. He reported rare episodes of vomiting. The CT examination revealed a large epigastric mass （8 cm × 4 cm） involving the lesser curvature of the stomach, in contact with the pancreas and gallbladder. Fatty areas within the mass were evident. A total gastrectomy together with cholecystectomy was performed. The histopathological diagnosis was a well differentiated liposarcoma. The patient did not undergo any adjuvant treatment, he is under close follow up and two years later he is disease free. We report this case due to the rarity of this tumor in the stomach （nine cases reported in the literature）.     

Giant primary liposarcoma of the stomach: a case report

Liposarcoma is the most common soft tissue sarcoma and accounts for 15%–20% of all mesenchymal malignancies. The tumor occurs most frequently in the limbs, retroperitoneum and rarely has a visceral location. We report a case of a gastric liposarcoma in a male patient who was admitted to hospital for abdominal discomfort and feeling fullness lasting for a month. The CT examination revealed a giant epigastric mass (36 cm × 34 cm) involving the wall of the stomach. A subtotal gastrectomy together was performed. The histopathological diagnosis was a myxoïd liposarcoma. The patient did not undergo any adjuvant treatment. He was under close follow up and nine months after surgery he represented with tumor recurrence. We report this case due to the rarity of this tumor in the stomach (fifteen cases reported in the literature).     

Synchronous renal cell carcinoma and adrenocortical carcinoma: a rare case report and clinicopathologic approach

A case of synchronous adrenocortical carcinoma (ACC) and renal cell carcinoma (RCC) has not yet been described in the English medical literature, to our knowledge. We report a first such case of adrenocortical and renal cell carcinomas occurring simultaneously in a 53-year-old male. He presented with history of vague abdominal pain. Ultrasound followed by a computed tomography (CT) scan and a magnetic resonance imaging (MRI) examination revealed a 6.4 cm left adrenal mass and a 3.5 cm right renal mass. The patient had complaints of gastroparesis manifesting with constant nausea as well as intermittent abdominal bloating and abdominal pain. He also had history of profuse intermittent sweating. There was no history of palpitations or fluctuations in blood pressure. The patient's urinary vanillylmandelic acid (VMA) levels and serum cortisol levels were normal. His 24-hour urine metanephrine levels were slightly elevated. Left adrenalectomy and right partial nephrectomy were performed. In this case, it is important to determine whether these tumors represent metastases or two synchronous tumors, as this has implications on the patient's management and prognosis. Clinical and pathological clues that led to the diagnosis are discussed in detail.     

Extragonadal retroperitoneal embryonal carcinoma successfully treated with chemotherapy

A 27-year-old Japanese man visited our hospital for further evaluation of multiple shadows on his chest X-ray. A 6 cm hard mass was palpable in the left lower abdominal region. Histological examination revealed that the lung tumor resected by the video-assisted thoracoscopic surgery was an embryonal carcinoma (EC). He was diagnosed as retroperitoneal EC with multiple lung metastases. He underwent chemotherapy with cisplatin, etoposide, and bleomycin, followed by 3 courses. A curative surgical operation revealed that there were no malignant cells in the lung lesions and primary lesion. This patient responded well to chemotherapy and achieved complete remission by chemotherapy.     

A case of watery diarrhoea syndrome due to an adrenal phaeochromocytoma secreting vasoactive intestinal polypeptide with coincidental autoimmune thyroid disease

A 40 year old woman presented with a 10 year history of watery diarrhoea and an acute quadriparesis. On clinical examination there was severe muscle weakness and a nodule was palpable in the thyroid gland. Biochemical testing revealed a hypokalaemia at 1.6 mmol/l. Plasma levels of VIP were raised at 202 pmol/l. CT scanning demonstrated a mass in the area of the left adrenal gland, and isotope scanning of the thyroid gland showed a 'cold' nodule. The plasma catecholamines and calcitonin were elevated. The patient also presented with psychiatric symptoms, and the relevance of these to her condition has been discussed. At operation a left adrenal tumour was removed. Post-operatively the patient's symptoms disappeared and the plasma hormone levels returned to normal values. Histological examination of the tumour revealed a well differentiated phaeochromocytoma which contained VIP and calcitonin. The thyroid nodule was excised and showed histological features of autoimmune thyroid disease. It is suggested that in all cases of the WDHA syndrome where the tumour is in an extra-pancreatic site patients should be screened for phaeochromocytoma.     

Mesonephric-like adenocarcinoma of the ovary: A case report and a review of the literature

Rationale:Mesonephric-like adenocarcinoma (MLA) from ovary is a very rare tumor which derives from mesonephric duct remnant of the female genital tract. Only six cases have been reported so far in the English literature.Patient concerns:A 29-year-old female patient was referred to the local hospital with a 20-day history of abdominal discomfort.Diagnoses:Pelvic ultrasound examination revealed a solid and cystic mass measuring 10 cm in diameter in the right adnexal area and a cystic mass measuring 5 cm in the left adnexal area. Postoperative pathology in the local hospital revealed suspected malignancy of the right ovary, and she was then transferred to our institution for definite diagnosis. The tumor mass was finally diagnosed as a primary MLA arising from the right ovary by histological and immunohistochemical examination in our institution.Interventions:The patient underwent laparoscopic right adnexectomy and removal of left ovarian cyst in the local institution. Then, she underwent a complete staging surgery including a total hysterectomy, left adnexectomy, pelvic plus para-aortic lymphadenectomy, and omentectomy in our hospital. In addition, she received four cycles of combination chemotherapy with carboplatin plus paclitaxel.Outcomes:There is no evidence of recurrence with 13 months of follow-up till now, and we are still following-up this patient.Lessons:MLA is an extremely uncommon malignancy with difficult diagnosis, unclear treatment and poor prognosis. Familiarizing with the clinical features and optimal management of this rare tumor may increase awareness of the disease among clinicians and pathologists, thus avoiding the misdiagnosis and mistreatment.     

Adenosquamous pancreatic cancer producing parathyroid hormone-related protein

An autopsy case of adenosquamous pancreatic cancer in a 61-year-old male patient with an elevated serum level of parathyroid hormone-related protein (PTH-rP) is reported. He was admitted to our hospital with a 1-month-long history of abdominal discomfort and progressive abdominal fullness. A computed tomography (CT) scan of the abdomen showed a retroperitoneal mass, approximately 10cm in diameter, involving the pancreas, with round enhancement on contrast examination. Histological examination of a specimen taken by CT-guided needle biopsy suggested squamous cell carcinoma or transitional cell carcinoma. Laboratory data on admission revealed a high serum calcium level and high PTH-rP level. The calcium level initially responded to intravenous hydration, furosemide, calcitonin, and bisphosphonates, decreasing from 15.0 to 9.0mg/dl. However, the hypercalcemia recurred after 10 days. The patient developed carcinomatous peritonitis and acute renal failure, and died on the 25th hospital day. Autopsy revealed a mass in the pancreatic body to tail, invading the retroperitoneum, with progressive carcinomatous peritonitis. Histological examination of the mass revealed infiltrating carcinoma, showing squamous differentiation with focal intracytoplasmic lumina formation, consistent with pancreatic adenosquamous carcinoma. Immunohistological examination showed positive staining for PTH-rP. Adenosquamous carcinoma of the pancreas is relatively rare; only a few cases associated with hypercalcemia and for which PTH-rP has been identified as a causative factor have been reported. This is the first case in which immunohistochemistry proved localized PTH-rP in adenosquamous pancreatic cancer cells, associated with persistent hypercalcemia.     

Renal cell carcinoma presenting with skull metastasis: a case report and literature review

The occurrence of metastasis to the head and neck region in renal cell carcinoma is extremely rare. An 80-year-old man presented with a soft nodule in the left parietal calvarium and was admitted to our hospital. Biopsy of the nodule showed nests of clear tumor cells, suggesting metastatic renal cell carcinoma. Computed tomography of the abdomen revealed a well-defined hypervascular tumor, measuring around 7 x 7 x 8 cm, exophytic from the lower pole of the right kidney. Since there were no other systemic metastases, right nephrectomy and complete resection of the skull lesion were performed. No adjunctive therapy was given postoperatively. After 22 months of follow-up, the patient was well and without evidence of disease.     

Actinomycosis of the thigh presenting as a soft-tissue neoplasm.

A 14-year-old boy presented with a mass measuring 6 cm x 9 cm, proximal to the patella on the anterior aspect of the knee. Magnetic resonance imaging revealed a 6 x 8 x 4 cm smoothly marginated slightly heterogeneous ellipsoidal mass which displaced musculature, the adjacent cutaneous fat and the quadriceps tendon. At surgery, the mass was noted not to penetrate the superficial surface of the quadriceps tendon and was full of slimy fluid. Histological examination revealed multiple basophilic lobulated granules with homogeneous centres and eosinophilic peripheral clubbed projections. The organisms grown anaerobically were identified as Actinomyces species. The anatomical site of the lesion is very unusual for actinomycosis and the exact route of infection in this patient remains unclear.     

Gastric sarcoidosis: Rare revealing feature of systemic sarcoidosis

Gastric sarcoidosis is clinically manifest in less than 1% of patients with systemic disease. Stomach is the most commonly involved site. Clinical signs are non-specific.We report the case of a female patient presenting with abdominal pain with no associated symptoms. Upper gastrointestinal endoscopy showed mucosal erosions. Biopsy and histological study revealed the presence of a gastric non-caseating granuloma. No other involvement was found and infectious granulomatosis was ruled out. Patient was treated with corticosteroids with substantial improvement. One year later, the patient presented with respiratory symptoms and joint pain. Chest computed tomography showed multiple mediastinal and bilateral hilar lymphadenopathies. It also highlighted the presence of interstitial lung disease and a 1 cm pulmonary nodule. Histological examination of this nodule revealed non-caseating granuloma. Serum angiotensin converting enzyme level was elevated. Lymphocytic alveolitis was diagnosed with bronchoalveolar lavage. Systemic sarcoidosis with gastric involvement was suggested. Patient was treated with corticosteroids and developed a favourable clinical course.Isolated gastric sarcoidosis is a challenging diagnosis. Histological findings are the key to diagnosis. Other granulomatous diseases need to be excluded especially infectious diseases before establishing the diagnosis of sarcoidosis. Corticosteroids are used in treating gastric sarcoidosis, yet no guidelines exist for the treatment of this rare condition.     

Disseminated form of Rosai-Dorfman disease. A case report

INTRODUCTION: Extranodal involvement in sinus histiocytosis with massive lymphadenpathy (SHML) or Rosai-Dorfman disease is common, seen in 43% of cases. We present a case of disseminated form of SHML with thyroid, renal, bone and lung involvement. EXEGESIS: A 53-year-old woman presented with cervical lymphadenopathy and a large palpable mass in the submandibular area. A thyroid nodule was palpable. Laboratory data showed an increase of the erythrosedimentation rate and a polyclonal hypergammaglobulinemia. Histopathological examination of a cervical lymph node biopsy showed typical features of SHML. CT scan showed enlargement mediastinal lymph node, mass of the right lung and infiltrative mass in the right renal hilium. Radiographs revealed lytic lesions in the right proximal tibia and left patella. Histopathology of a biopsy from the kidney mass and thyroidectomy displayed typical characteristics of SMHL. The patient was treated by steroids with a markedly regression of the kidney and renal masses. CONCLUSION: Multiple extranodal involvement in SMHL is rare, the prognosis may be poor when lesions are massive and involve vital organs.