Primary retroperitoneal teratomas involving adrenal glands are exceedingly uncommon accounting for only 4% of all primary teratomas. They are more common in childhood and rarely occur in adults. Only a very few case reports have been documented in literature so far. Herein, we report a mature (benign) cystic retroperitoneal teratoma in the region of left adrenal gland in a 22-year-old otherwise healthy male patient who presented with a 1-month history of left flank pain. In addition, a literature review on teratomas is included.
Soft tissue necrotizing infections are a significant cause of morbidity and mortality. The aim of this study is to present a patient with necrotizing infection of abdominal wall resulting from the rupture of a retroperitoneal stromal tumor.
PRESENTATION OF CASE
We present a 60-year-old Caucasian male patient with necrotizing infection of abdominal wall secondary to the rupture of a retroperitoneal stromal tumor. The patient was initially treated with debridement and fasciotomy of the anterior abdominal wall. Laparotomy revealed purulent peritonitis caused by infiltration and rupture of the splenic flexure by the tumor. Despite prompt intervention the patient died 19 days later. The isolated microorganism causing the infection was the rarely identified as cause of infections in humans Pediococcus sp., a gram-positive, catalase-negative coccus.
Necrotizing infections of abdominal wall are usually secondary either to perineal or to intra-abdominal infections. Gastrointestinal stromal cell tumors could be rarely complicated with perforation and abscess formation. In our case, the infiltrated by the extra-gastrointestinal stromal cell tumor ruptured colon was the source of the infection. The pediococci are rarely isolated as the cause of severe septicemia.
Ruptured retroperitoneal stromal cell tumors are extremely rare cause of necrotizing fasciitis, and before this case, Pediococcus sp. has never been isolated as the responsible agent.
Necrotizing inflammation; Gangrene Fournier; Pediococcus; Fasciitis
A 48-year-old male who presented with an enlarged right scrotum was diagnosed with malignant transformation of testicular teratoma. Physical examination revealed a right scrotal mass of hard consistency with no inguinal lymphadenopathy. Since prepuberty, his right testis had been larger than the left one, with no pain or tenderness. Computed tomography and bone scan revealed retroperitoneal lymphadenopathy and multiple bone metastases. Right orchiectomy was performed immediately, and a pathological examination revealed a mature teratoma associated with adenocarcinoma, showing signet ring cell differentiation. Cisplatin-based combination chemotherapy was administered; however, the metastatic lesions progressed, and the patient succumbed to the disease after 15 months. Only a few cases of primary malignant transformation of teratoma in the testis have been reported, and this is the first case report of primary malignant transformation of teratoma in the testis with signet ring cell-type differentiation.
adenocarcinoma; testis; teratoma
In the literature, 51 cases of primary retroperitoneal mucinous cystadenocarcinoma have been published. We report the fourth case occurring in a male patient. The 42-year-old patient presented with multiple retroperitoneal cystic masses causing abdominal discomfort without alteration of the global clinical state. The masses were totally removed by a two-stage surgery. No other treatment has been introduced. After a follow-up of 6 months, the patient is disease-free. This rare tumor most likely arises from the mucinous metaplasia of peritoneal inclusion cysts rather than from ectopic ovarian tissue or ovarian teratomas. The occurrence of such a tumor in a male patient supports this theory. Preoperative diagnosis is mostly difficult. Clinical behavior and treatment are still controversial.
Teratomas are the commonest germ cell tumours and are most frequently found in the testes and ovary. Extragonadal teratomas are rare and mainly occur in midline structures. Uterine teratomas are extremely rare with only a few previous case reports, usually involving mature teratomas of the uterine cervix.
We report an 82-year-old lady presenting with post-menopausal bleeding. Initial investigations revealed a benign teratoma of the uterus which was removed. Her symptoms persisted and a recurrent, now malignant, teratoma of the uterine corpus was resected at hysterectomy. Six months after surgery she relapsed with para-aortic lymphadenopathy and was treated with a taxane, etoposide and cisplatin-containing chemotherapy regimen followed by retroperitoneal lymph node dissection.
In this report we discuss the aetiology, diagnosis and management of uterine teratomas, and review previous case studies.
The retroperitoneum is an uncommon location for teratoma in adults. The current study presents the case of a rare giant primary retroperitoneal teratoma in a 55-year-old female. The clinical manifestations, diagnosis and surgical treatment procedure of this case are retrospectively reviewed. The patient presented with a complaint of an abdominal palpable mass and fullness for 1 month. The patient suffered a massive hemorrhage during the first exploratory laparotomyand only a small section of the tumor was resected. Pathology revealed a mature retroperitoneal teratoma. Eleven months after the first surgery, the tumor was resected successfully at the second laparotomy. The surgical specimen was a large tumor mass measuring 22×18×10 cm in size and weighing 6 kg. At follow-up, the patient was in a stable condition. This case study highlights the importance of imaging for the development of a pre-operative strategy and performing a safe surgical excision in giant retroperitoneal teratoma cases.
retroperitoneal teratoma; primary; adult
Teratomas are neoplasms that arise from pluripotent cells and can differentiate along one or more embryonic germ lines. Renal teratoma is an exceedingly rare condition. Teratomas commonly arise in the gonads, sacrococcygeal region, pineal gland, and retroperitoneum. They present mainly as an abdominal mass with few other symptoms. Majority of the tumors are benign, situated on the left side and para renal, occasional lesions are bilateral. If diagnosed early, they are amenable to curative excision.
Renal teratomas are rare and most have been dismissed as cases of teratoid nephroblastomas or retroperitoneal teratomas secondarily invading the kidney. The differentiation between these two neoplasms in the kidney is often problematic.
We present a case of intrarenal immature teratoma in a six-month-old baby girl.
The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1746249869599954.
Teratoma; Immature; Wilms; Kidney; Extragonadal
Retroperitoneal cysts are rare, usually asymptomatic, abdominal lesions. Epidermoid cysts developing in this space usually occur in middle-aged women and are incidentally discovered in the presacral region during ultrasound examination. Occasionally, cysts may arise from splenic tissue or adrenal glands and develop above the presacral area.
PRESENTATION OF CASE
We present the unusual location of a cyst in the retroperitoneal space in a 41-year-old woman admitted to hospital due to detection of a lesion in ultrasound imaging. A CT scan confirmed large intra-abdominal cyst. At operation, a large retroperitoneal thin-walled cyst with no evident arising point was discovered. Histologic analysis revealed epidermoid cyst.
Our patient presented with giant retroperitoneal cyst extending from the subhepatic region to the uterine and bladder. To our knowledge, this unusual location in adult has not been previously reported in the literature. In our case the lesion was adjacent to inferior vena cava and mesenteric vessel which required special attention during preparation and was technically demanding.
Surgery is the gold standard for the diagnosis and treatment of retroperitoneal epidermoid cysts. Successful treatment of benign retroperitoneal epidermoid cysts depends on appropriate diagnosis, careful operative technique, and adequate management of the underlying pathology.
Retroperitoneal space; Epidermoid cyst; Surgery
The lung and stomach are very unusual sites for teratoma. The histologic findings of intrapulmonary and gastric teratomas are not different from those arising in usual sites, such as the ovary or testis. However, preoperative diagnosis is sometimes difficult to make partly because of unusual location. We report here two cases of teratoma, one intrapulmonary teratoma and the other gastric. The intrapulmonary teratoma in our study had an endobronchial tumor growth, which rules out mediastinal teratoma. Meanwhile gastric teratomas usually present as a submucosal tumor and most cases are reported in infancy and childhood. Gastric teratoma in this study occurred in a 27-year-old man. To the best of our knowledge, this case of intrapulmonary teratoma is the eighth and the gastric teratoma is the first to be reported in Korea.
Retroperitoneal necrotizing fasciitis is a rare, fulminant, and potentially lethal complication of intra-abdominal suppuration. A retroperitoneal origin is very rare and very few cases have been reported in the literature. To the best of our knowledge, this case is only the fourth case reported of successful management following retroperitoneal necrotizing fasciitis.
A 33-year-old Tamang man presented to our facility with a history of five days of fever and vomiting and eight days of severe left loin pain. On examination, he had features of peritonism. A laparotomy was performed, revealing extensive necrotizing fasciitis of the retroperitoneum extending to the anterior abdominal wall. Our patient survived following extensive debridement of the necrotic tissues and supportive care.
Retroperitoneal necrotizing fasciitis can rarely present with features of peritonism, and hence should be included as a possible differential diagnosis for anyone presenting with peritonism. Although a fatal condition, early intervention and aggressive management can save the life of a patient.
Teratomas are bizarre neoplasms derived from embryonic tissues that are typically found only in the gonadal and sacrococcygeal regions of adults. Retroperitoneal teratomas are rare and present challenging management options. We report here the case of a histologically unusual retroperitoneal tumor detected on computed tomography during the workup of abdominal pain in a 32-year-old male. The evaluation and treatment of this condition and a review of the literature are included in this paper.
Cystic ovarian teratomas comprise 20% of all ovarian neoplasms, and are commonly encountered in patients between 20 and 40 years of age. Although these cysts are usually asymptomatic, we present the case of a patient whose cyst resulted in pruritus and abdominal pain. Based on a MEDLINE search of the literature, we believe this is the first case report of a twisted ovarian cyst presenting with generalized pruritus.
A 35-year-old Sri Lankan woman presented with lower abdominal pain of one day’s duration with vomiting and generalized pruritus. She had no history of allergies and was not on medication. Upon a physical examination, our patient was found to have an acute abdomen, localized peritonism in her lower abdomen and tachycardia of 100 beats per minute. Computed tomography showed that the cyst, which contained calcified structures, originated from her left ovary. After laparoscopy-assisted removal of the twisted ovarian cyst, her symptoms resolved completely. Histological examination confirmed a benign ovarian teratoma.
An unusual case of torsion of an ovarian teratoma presenting with abdominal pain and generalized pruritus, believed to be due to an antibody-mediated response, was resolved after surgical removal of the cyst.
Adrenal gland pseudocysts are not common conditions, and most of them are nonfunctional and asymptomatic. However, large pseudocysts may causes abdominal discomfort and have compressive effects on adjacent organs. They may rupture spontaneously or after trauma, and lead to retroperitoneal hemorrhage and surgical emergency. Herein, we report a case of 21-year-old female who presented with acute abdomen and hemorrhagic shock due to spontaneous rupture of adrenal pseudocyst. She was treated successfully by open surgery, removal of adrenal pseudocyst and unilateral adrenalectomy.
Adrenal; pseudocyst; adrenal cyst; hemorrhagic shock
A 34-year-old female presented with sudden onset of severe abdominal pain in a flank distribution. A large mass was palpable in the right upper quadrant on physical examination. Abdominal contrast-enhanced computed tomography showed a well-defined, right-sided, retroperitoneal cystic lesion located between the abdominal aorta and the inferior vena cava (IVC). The tumor size was 55 × 58 mm, and it compressed the gallbladder and the duodenum. Upper gastrointestinal radiography revealed a stricture of the second portion of the duodenum by the tumor. T2-weighted magnetic resonance imaging showed that the whole part was hyperintense with hypointense rims, but the inner was partially hypointense. Based on the radiological findings, the preoperative differential diagnosis included retroperitoneal teratoma, Schwannoma, abscess, and primary retroperitoneal tumor. On laparotomy, the tumor was located in the right retroperitoneal cavity. Kocher maneuver and medial visceral rotation, which consists of medial reflection of the upper part of right colon and duodenum by incising their lateral peritoneal attachments, were performed. Although a slight adhesion to the IVC was detected, the tumor was removed safely. Thin-section histopathology examination detected neither tumor tissues nor any tissues such as adrenal gland, ovarian tissue, or endometrial implants. The final pathological diagnosis was idiopathic retroperitoneal hematoma; the origin of the bleeding was unclear. The patient was discharged without any complication 5 days after the operation.
Retroperitoneal cystic tumor; Idiopathic hematoma; Chronic expanding hematoma
Acute appendicitis may occasionally become extraordinarily complicated and life threatening yet difficult to diagnose. One such presentation is described in a 60-year-old man who was brought to the hospital due to right lumbar pain and fever for the last 15 days. Ultrasonography showed a right perinephric gas and fluid collection. Abdominal computed tomography with multidetector-row CT (MDCT) revealed gas-containing abscess in the right retroperitoneal region involving the perinephric space, extending from the lower pole of the right kidney up to the bare area of the liver. Inflamed retrocecal appendix was seen on thick multiplanar reformat images with its tip at the lower extent of the abscess. Laparotomy and retroperitoneal exploration were performed immediately and a large volume of foul smelling pus was drained. A ruptured retrocecal appendix was confirmed as the cause of the abscess.
Perinephric abscess; retrocecal appendicitis; multidetector-row CT-MDCT
Elevated intra-abdominal pressure (IAP) has been identified as a cascade of pathophysiologic changes leading in end-organ failure due to decreasing compliance of the abdomen and the development of abdomen compartment syndrome (ACS). Spontaneous retroperitoneal hematoma (SRH) is a rare clinical entity seen almost exclusively in association with anticoagulation states, coagulopathies and hemodialysis; that may cause ACS among patients in the intensive care unit (ICU) and if treated inappropriately represents a high mortality rate.
We report four patients (a 36-year-old Caucasian female, a 59-year-old White-Asian male, a 64-year-old Caucasian female and a 61-year-old Caucasian female) that developed an intra-abdominal hypertension due to heparin-induced retroperitoneal hematomas after implantation of ventricular assist devices because of heart failure. Three of the patients presented with dyspnea at rest, fatigue, pleura effusions in chest XR and increased heart rate although b-blocker therapy. A 36-year old female (the forth patient) presented with sudden, severe shortness of breath at rest, 10 days after an "acute bronchitis". At the time of the event in all cases international normalized ratio (INR) was <3.5 and partial thromboplastin time <65 sec. The patients were treated surgically, the large hematomas were evacuated and the systemic manifestations of the syndrome were reversed.
Identifying patients in the ICU at risk for developing ACS with constant surveillance can lead to prevention. ACS is the natural progression of pressure-induced end-organ changes and develops if IAP is not recognized and treated in a timely manner. Failure to recognize and appropriately treat ACS is fatal while timely intervention - if indicated - is associated with improvements in organ function and patient survival. Means for surgical decision making are based on clinical indicators of adverse physiology, rather than on a single measured parameter.
Development of a sarcomatous component in a germ cell tumor is an uncommon phenomenon. Most cases reported have a grim prognosis. Growing teratoma syndrome is also an uncommon phenomenon and occurs in approximately 2% to 7% of non seminomatous germ cell tumors and should be treated surgically.
We report the case of a 12-year-old Asian girl with an ovarian mixed germ cell tumor containing a rhabdomyosarcomatous component. She was treated with a germ cell tumor chemotherapy regimen and rhabdomyosarcoma-specific chemotherapy. Towards the end of her treatment, she developed a retroperitoneal mass that was increasing in size. It was completely resected, revealing a mature teratoma, consistent with growing teratoma syndrome. She is still in complete remission approximately three years after presentation.
The presence of rhabdomyosarcoma in a germ cell tumor should be treated by a combined chemotherapy regimen (for germ cell tumor and rhabdomyosarcoma). In addition, development of a mass during or after therapy with normal serum markers should raise the possibility of growing teratoma syndrome that should be treated surgically.
Retroperitoneal schwannomas are rare, usually benign tumors that originate in the neural sheath and account for only a small percentage of retroperitoneal tumors. The aim of this clinical study is to present our experience in managing retroperitoneal schwannomas with a review of the current literature and to point out the surgical technical difficulties we faced, due to the tumor's strange behavior that eroded the vertebra in two cases without causing malignant invasion.
We reviewed the medical files of 69 patients treated in our department for retroperitoneal tumors from January 1991 until December 2006. Five patients had retroperitoneal schwannomas according to pathology report.
There were two male and three female patients, with a mean age of 56 years (range 44–67 years). All patients were asymptomatic and none suffered from von Recklinghausen disease. Imaging workup included ultrasonography, computed tomography and magnetic resonance imaging. One patient, after having a non-diagnostic computed tomography fine needle aspiration (CT-FNA), underwent exploratory laparotomy and incisional biopsy that established the diagnosis of schwannoma. After complete excision of the tumors, postoperative course was uneventful in all patients. Tumors' maximum diameter was 12.7 cm (range 7–20 cm). No recurrences were detected during the follow up period (6–75 months).
Preoperative establishment of diagnosis is difficult in case of retroperitoneal schwannomas, however close relationship of retroperitoneal tumors with adjacent neural structures in imaging studies should raise a suspicion. Complete surgical resection is the treatment of choice. Histology and Immunohistochemistry confirms the diagnosis.
The morphological features of a retroperitoneal teratoma in a 10-month-old girl are reported. Unlike the usual pattern of the teratoma, this tumor was composed predominantly of nephroblastomatous tissue. Histologically, glomeruloid and tubular structures were identified in nests of undifferentiated blastemal elements. Hyaline cartilage, adipose tissue, glial tissue and glands lined by mucin-secreting columnar epithelium were minor elements. A focal cystic structure lined by thin flattened epithelium was also noted. Retroperitoneal teratoma with predominance of nephroblastic elements is of interest not only because of its rarity but also because it needs to be differentiated from extrarenal Wilms' tumor, since both of these tumors have different origins.
Gastric teratomas are very rare tumours in children. They usually present with a palpable mass in the upper abdomen. We report a case of gastric teratoma in one and half month old male infant who presented with a palpable mass in abdomen, extending from epigastrium to the pelvis. Ultrasound of abdomen revealed a huge mass with solid and cystic components. CT scan delineated calcifications in the mass. The preoperative diagnosis was a teratoma but not specifically gastric one. The mass was excised completely with seromuscular layer of the stomach wall. The histopathology confirmed it to be grade-3 immature gastric teratoma. The rarity of the origin of teratoma in addition to its immature variety prompted us to report the case.
Gastric teratoma; Immature teratoma; Infant
Retroperitoneal hemorrhagic bleeding is a known manifestation of Type-IV Ehlers-Danlos Syndrome that is caused by loss-of-function mutations of the pro-alpha-1 chains of type III pro-collagen (COL3A1) resulting in vascular fragility. A number of previous reports describe futile surgical intervention for retroperitoneal bleeding in Type-IV Ehlers-Danlos Syndrome with high post-operative mortality, although the rarity of retroperitoneal bleeding associated with Type-IV Ehlers-Danlos Syndrome precludes an evidence-based approach to clinical management. We report a 23-year-old male with history of Type-IV Ehlers-Danlos Syndrome who presented with severe abdominal pain and tachycardia following an episode of vomiting. Further work-up of his abdominal pain revealed massive retroperitoneal bleeding by CT-scan of the abdomen. Given numerous cases of catastrophic injury caused by surgical intervention in Type-IV Ehlers-Danlos Syndrome, the patient was treated non-operatively, and the patient made a full recovery. This case suggests that even in cases of large retroperitoneal hemorrhages associated with Ehlers-Danlos Syndrome, it may not truly represent a surgical emergency.
Type-IV Ehlers-Danlos Syndrome; retroperitoneal hemorrhage; conservative management; non-operative; COL3A1; connective tissue disorder.
A 59-year-old man presented with abdominal and left flank pain. The symptom had started 30 days before as an acute nephrolithiasis, which had worsened despite conservative management. The abdomen was slightly distended and tender over the lower abdomen, without signs of generalized peritoneal irritation. A computed tomography (CT) scan showed an abscess in left para-renal space up to the subphrenic space and an unexpected pneumomediastinum. An emergency operation was performed, which showed retroperitoneal diverticulitis perforation of the sigmoid descending junction with abscess formation. A segmental resection of the diseased colon and end-colostomy was performed (Hartmann's procedure). However, the patient's condition progressively deteriorated, and he died of sepsis and multi-organ failure on the 5th postoperative day. Although pneumomediastinum caused by colonic diverticulitis perforation is extremely rare, it could be a life-threatening condition in patients without signs of peritonitis because of delayed diagnosis.
Pneumomediastinum; Diagnostic; Diverticulitis; Colonic; Perforation
Duodenal rupture secondary to blunt trauma is a relatively uncommon event and is usually a result of a road traffic accident. As the duodenum is a retroperitoneal organ, delays in diagnosis can occur, as the patient may present with vague abdominal symptoms and other non-specific signs. Computed tomographic scanning is therefore a useful tool in the diagnosis of this condition. We present a 19-year-old girl who was hit in the abdomen with a football and subsequently had a duodenal rupture.
Retroperitoneal teratoma with malignant transformation is a rare condition in adults. Its most common malignant transformation is into a squamous cell carcinoma, but rarely into a mucinous adenocarcinoma. Postoperative treatment of mucinous adenocarcinoma arising from teratomas has not been established due to its rare incidence. Here we present a case of retroperitoneal mucinous adenocarcinoma arising from a teratoma in the presacral area. Operative and postoperative managements are described with a brief review of the literatures.
Retroperitoneal neoplasms; Teratoma; Mucinous cystadenocarcinoma; Chemoradiotherapy
Late relapse of a testicular cancer is an uncommon occurrence. We report a case of late relapse of a testicular tumour combined with a renal cancer and their successful removal with retroperitoneoscopy. The 36-year-old patient underwent left orchiectomy, retroperitoneal lymph node dissection, and chemotherapy, because of mixed tumor including teratoma and embryonal carcinoma. 18 years after the successful primary therapy elevated serum alpha-fetoprotein level had been confirmed, then MRI and PET-CT scans demonstrated a 30 mm left renal mass and 22 mm retroperitoneal lymph node above the bifurcation of the left common iliac artery. We performed retroperitoneoscopic lymph node dissection and left renal tumour resection in the same session. The histology revealed embryonal carcinoma for the retroperitoneal lymph node and renal cell carcinoma for the left renal mass. We can conclude that late followup of patients with testicular tumour is important. Retroperitoneoscopy is feasible approach for the removal of retroperitoneal lymph node metastasis and resection of renal tumor.