A puzzling ovarian tumour: pregnancy luteoma with diffuse endometriosis.

BACKGROUND: Pregnancy luteoma is a distinctive non-neoplastic hormone dependent lesion arising in pregnancy and mimicking an ovarian tumour. Fewer than 200 cases have been described in the English-language literature. Its clinical and morphological features are characteristic and must be considered in order to prevent diagnostic misinterpretation. To the best of our knowledge the association of pregnancy luteoma with endometriosis has not been reported in literature to date. CASE REPORT: A 30-year-old pregnant woman with no particular past medical history, consulted her gynaecologist at 17 weeks gestation for routine check-up. The patient was asymptomatic and did not show any signs of virilization. Ultrasonography disclosed a left adnexal heterogeneous mass measuring 7 cm in diameter with intramural vegetations. The right ovary was unremarkable. The patient underwent salpingo-oophorectomy considering the imaging findings were suspicious for malignancy. Histologically, the lesion was constituted of large sheets of luteinized polygonal cells with abundant eosinophilic cytoplasm and small round nuclei devoid of atypia and mitotic figures. In addition, there were several ectopic endometrial glands surrounded by abundant decidualized or edematous stroma. Immunohistochemically, these glands were immunoreactive for cytokeratin 7. The final pathological diagnosis was pregnancy luteoma associated with diffuse endometriosis. CONCLUSIONS: Because of its relative rarity, pregnancy luteoma is likely to be clinically misinterpreted and overtreated, as in the present case.

Jejunal lymphangioma: an unusual cause of intussusception in an adult patient.

Adult intussusception is a relatively rare clinical entity. Almost 90% of cases of intussusception in adults are secondary to a pathologic condition that serves as a lead point. Lymphangioma of the small bowel is an unusual tumour that has been rarely reported to cause intussusception. In this paper, we present a rare case of adult intussusception due to jejunal lymphangioma. A 22-year-old female patient with a medical history significant for anaemia presented with intermittent colicky abdominal pain, diarrhoea and oedema of the inferior limbs for the past three months. Ultrasonography and CT scan revealed a typical target sign with dilated intestinal loops. At laparotomy, a jejuno-jejunal intussusception was found. Partial resection of the jejunum was performed. Macroscopic examination of the surgical specimen revealed a pedunculated polyp measuring 2 cm in diameter. Histological sections of the polyp revealed in the lamina propria and submucosal layer of the jejunum several markedly dilated thin-walled lymphatic spaces lined with single layers of flat endothelial cells. The final pathologic diagnosis was submucosal lymphangioma. This case report indicates that intussusception, although rare in adults, should be considered in the differential diagnosis of abdominal pain. Moreover, it should be taken into consideration that lymphangioma is one of the possible lesions that can cause intussusception.

Neuroendocrine tumours of the pancreas: a clinicopathological study of nine cases including six insulinomas.

BACKGROUND: Pancreatic neuroendocrine tumours (pNET) are relatively uncommon, accounting for 1-2% of all pancreatic neoplasms. They are characterised by varying clinical presentation, tumour biology and prognosis. AIM: To provide an updated overview on clinicopathological features, treatment and outcome of pNET. PATIENTS AND METHODS: In our retrospective study, we reviewed 9 cases of pNET that were diagnosed at the Pathology Department of Mongi Slim Hospital over an 11-year period (2003- 2013). Relevant clinical information and microscopic slides were available in all cases and were retrospectively reviewed. The latest WHO classification (2010) was adopted. RESULTS: Our study group included 3 men and 6 women (M/F ratio 0.5) with an age between 20 and 75 years (mean = 52 years). Pancreatic neuroendocrine tumours ranged in size from 0.5 to 10 cm (mean 4 cm). The sites of pNET were the head of the pancreas (n = 4), the body of the pancreas (n = 3) and the tail of the pancreas (n = 2). Enucleation of the tumour was performed in five cases, Three patients underwent distal pancreatectomy and splenectomy, whereas only one patient had central pancreatectomy. Histopathological examination of the surgical specimen coupled with immunohistochemical study established a diagnosis of pNET grade 1 (G1) in seven cases and grade 2 (G2) in two cases. CONCLUSION: Pancreatic neuroendocrine tumours are a heterogeneous group of neoplasms with distinct tumour genetics, biology and clinicopathological features. Accurate clinical and pathologic diagnosis is an important first step in developing an appropriate management plan.

Hydatid cyst presenting as a breast lump in a male patient.

The breast is a rare primary site of hydatid disease and accounts for only 0.27% of cases. Mammary hydatidosis generally occurs in females and has never been described in male patients. In this paper, the authors report a new case of isolated hydatid cyst of the breast in a 35-year-old previously healthy man, who presented with a left breast painless lump of one year duration. Physical examination showed a non-tender and immobile mass in the upper lateral quadrant of the left breast, with normal overlying skin and nipple. There was no palpable lymph node in the left axilla and the contralateral breast was normal. Ultrasonography showed a 2.7 x 1.5 cm cystic lesion in the left breast. The patient underwent total excision of the mass, and histopathological examination confirmed the diagnosis of hydatid cyst. The authors conclude that although hydatid cyst of the breast is rare, it should be considered in the differential diagnosis of breast lumps especially in endemic areas.

Sclerosing stromal tumour of the ovary: two case reports.

Sclerosing stromal tumours are rare benign ovarian neoplasms of the sex cord stromal that occur predominantly in the second and third decades of life. Herein, we report two cases of sclerosing stromal tumour of the ovary. The two patients were 16 and 45 years old and both presented with pelvic pain. Ultrasonography demonstrated a heterogeneous solid mass of the left and right ovary, respectively, with some cystic foci in the second tumour. Laboratory tests including tumour markers and serum hormonal assays were normal in both cases. The two patients underwent left and right salpingo-oophrectomy, respectively. Microscopically, the tumours showed a pseudolobular pattern with cellular areas separated by oedematous and collagenous areas. The cellular areas were richly vascularized, with a hemangiopericytic pattern, and were composed of an admixture of theca-like and spindle-shaped cells. Immunohistochemical studies showed that the tumour cells were positive for smooth muscle actin, inhibin and vimentin, but negative for cytokeratin. The final pathological diagnosis was sclerosing stromal tumour. Postoperative course was uneventful for both patients.

Pancreatic heterotopia of the small intestine: two case reports.

The presence of heterotopic pancreas is unusual with an estimated incidence of 0.2% of upper abdominal operations. Heterotopic pancreas occurs predominantly in the stomach, duodenum and proximal jejunum. Isolated pancreatic heterotopia of the ileum is very rare and is usually found in a Meckel's diverticulum. In most cases, these heterotopias are asymptomatic and are only incidentally detected upon pathological examination or autopsy. In this paper, the authors report two cases of pancreatic heterotopia involving, respectively, the duodenum and ileum that were fortuitously discovered on a surgical specimen and during laparotomy for unrelated causes.

Coexistence of xanthogranulomatous cholecystitis and gallbladder adenocarcinoma: a fortuitous association?

Xanthogranulomatous cholecystitis is a relatively uncommon variant of chronic cholecystitis, characterized by marked thickening of the gallbladder wall and dense local adhesions. Not only does xanthogranulomatous cholecystitis mimic malignancy, it can also be infrequently associated with gallbladder carcinoma in 0.2% to 35.4% of cases. Herein, the authors report a new case of xanthogranulomatous cholecystitis concomitant with gallbladder adenocarcinoma in a 65-year-old female patient. Because of its overlapping clinical, radiological and macroscopic findings with gallbladder cancer, definitive diagnosis of xanthogranulomatous cholecystitis relies on extensive sampling and thorough microscopic examination of the surgical specimen to exclude the possibility of coexisting tumour. It is still a matter of debate whether xanthogranulomatous cholecystitis is truly a precursor of gallbladder carcinoma or if it is just an incidental finding. This aspect needs to be explored in the future with further studies.

Coexistence of lobular granulomatous mastitis and ductal carcinoma: a fortuitous association?

A 77-year-old female patient with a medical history significant for hypertension and epilepsy presented with right breast pain of 6-months duration. Examination revealed a hard sub-areola tender mass with irregular borders associated with mild right nipple retraction. Mammography showed a 2.2 x 2.4 cm stellate mass of the right breast. Ultrasound-guided core biopsies of the tumour were performed. Pathological examination revealed a grade II infiltrating ductal carcinoma. The patient underwent right radical mastectomy with homolateral axillary lymphadenectomy. Histological examination of the surgical specimen revealed grade II infiltrating ductal carcinoma concomitant with granulomatous lobular mastitis. To the best of our knowledge, the coexistence of granulomatous lobular mastitis and ductal carcinoma has been described only twice in the English language literature. The theory that chronic inflammation leads to cancer is well documented. Whether our patient had developed cancer from granulomatous lobular mastitis or otherwise is a matter of debate until more cases are encountered and more research is done in the area of breast cancer pathogenesis with regards to it arising from granulomatous lobular mastitis.

Adrenal epithelial cyst: a case report.

Epithelial-lined or true cysts of the adrenal gland are exceedingly rare accounting for only 9% of all adrenal cysts. A 55-year-old woman with a medical history for cholecystectomy and liver hydatidosis presented with an 8-month history of abdominal pain in the upper left quadrant. Physical examination was unremarkable and laboratory tests were within normal range. Computed tomography displayed a homogeneous and finely calcified cystic mass of the left adrenal gland measuring 12 x 10 cm. A hydatid cyst of the adrenal gland was suspected preoperatively and the patient underwent uneventful excision of the cyst. Histological examination of the surgical specimen revealed that the cyst wall was lined by cuboidal to flattened cells with bland cytologic features. Immuohistochemically, the cyst lining expressed intensely cytokeratin but was negative for CD 34 and calretinin. The final pathological diagnosis was epithelial adrenal cyst.

Idiopathic granulomatous mastitis mimicking breast cancer: report of two cases.

Idiopathic granulomatous mastitis is a rare inflammatory breast disease of unknown aetiology that is frequently mistaken for breast carcinoma both clinically and mammographically. In this paper, the authors report two cases of idiopathic granulomatous mastitis that occurred in two parous women aged 38 and 45 years. Clinically, both patients presented with a tender palpable lump in the left breast. Mammography showed an poorly-defined mass in both patients with microcalcification in the first case and skin retraction in the second case. Breast lumpectomy was performed in both patients. Histological examination of the surgical specimen revealed non-caseating granulomas confined to breast lobules. Special staining for fungi and tuberculosis were all negative. Correct diagnosis ofidiopathic granulomatous mastitis requires the exclusion of malignancy, other granulomatous disease and infectious aetiologies. Histopathologic examination remains the gold standard for diagnosis. This disease is rare, and therefore the optimum treatment protocol is still being established.

Localized ileal giant pseudopolyposis in Crohn's disease: a case report.

Localized giant pseudopolyposis is a rare complication in inflammatory bowel disease defined as a pseudopolyp (isolated or clustered) larger than 1.5 cm in size. Giant pseudopolyps are more commonly found in ulcerative colitis compared to Crohn's disease and mainly involve the left colon. A 26-year-old male patient with a two-year history of Crohn's disease was admitted with increasing abdominal pain, vomiting, anorexia, weight loss and fever. On physical examination, the abdomen was diffusely tender. Computed tomography showed diffuse irregular thickening of the ileal wall and stenosis of the terminal ileum. The patient underwent ileo-cecal resection with re-anastomosis. The ileal portion of the resected specimen harboured multiple finger-like pedunculated polyps, with the smallest measuring 0.5 cm and the largest measuring 1.8 cm. Histologically, the polyps were consistent with granulation tissue. No evidence of dysplasia or malignancy was found. The post-operative course was uneventful considering one month follow-up. This report illustrates an unusual case of giant pseudopolyposis involving the ileum in a patient with Crohn's disease. The natural history of these lesions, as well as their optimal management, remain uncertain.

Pancreatic heterotopia in the gallbladder neck associated with chronic cholecystitis.

The gallbladder is an unusual location of pancreatic heterotopia defined as the presence of pancreatic tissue lacking anatomical and vascular continuity with the main body of the gland. A 55-year-old previously healthy male patient presented with repeated attacks of right hypochondriac pain and vomiting. On physical examination, the right upper abdomen was tender to palpation with a positive Murphy's sign. Abdominal ultrasonographic examination showed multiple gallstones within a thin-walled gallbladder. Laparoscopic cholecystectomy was performed with uneventful recovery. Macroscopic examination of the surgical specimen revealed a yellowish intramural nodule measuring 7 mm close to the neck of the gallbladder. Histological examination revealed chronic cholecystitis and subserosal foci of heterotopic pancreas made up of exocrine acinar and ductal elements without islet cells corresponding to incomplete heterotopia. Heterotopic pancreas is usually detected as an incidental microscopic finding in a gallbladder specimen removed by cholecystectomy. Pre-operative diagnosis is difficult primarily due to its non-specific clinical features.

Adenomatous transformation in a giant solitary Peutz-Jeghers-type hamartomatous polyp.

Solitary Peutz-Jeghers-type polyp is a rare hamartomatous polyp without associated mucocutaneous pigmentation or a family history of Peutz-Jeghers Syndrome. It is usually encountered in the small intestine, but rarely involves the rectum. A 27-year-old previously healthy female patient presented with a two-month history of rectal bleeding. The patient had neither mucocutaneous pigmentation nor a family history of gastro-intestinal polyposis. Endoscopic examination revealed a solitary lobular polypoid lesion in the lower rectum. The polyp was sessile and measured 15 cm in diameter. As histological examination of the biopsy specimen was suggestive of adenoma, endoscopic polypectomy was performed. Histologically, this polyp had an arborizing muscular network originating from the muscularis mucosa, and was covered by well organized mucosa with several foci of dysplastic glands. The final pathological diagnosis was solitary Peutz-Jeghers type hamartomatous polyp with adenomatous transformation.

Extraventricular neurocytoma in a child mimicking oligodendroglioma: a diagnostic pitfall.

Extraventricular neurocytomas are rare neuronal tumours that have been included in the 2007 WHO classification as a variant of central neurocytoma. They arise outside the ventricles, usually within the cerebral hemisphere,s but also in other regions throughout the neuraxis. The morphological overlap of these tumours with oligodendroglioma often poses diagnostic difficulty. Herein, a case of extraventricular neurocytoma in a 4-year-old girl is reported that mimicked histologically oligodendroglioma. The authors describe the clinicopathological features of this rare entity with special emphasis on differential diagnosis.

[Epidemiology of extrapulmonary hydatid cysts: 265 Tunisian cases]. / Epidémiologie des kystes hydatiques extrapulmonaires : 265 cas en Tunisie.

AIM OF STUDY: The authors wanted to analyze the epidemiological features of extrapulmonary hydatid cysts and compare their results with those reported in literature. METHODS: Two hundred and sixty-five cases of extrapulmonary hydatid cysts collected from 1990 to 2007 were retrospectively studied. RESULTS: One hundred and one male and 164 female patients (sex ratio M/F=0.61 mean age 38.7 years) were included. In our series, hydatid cysts involved mainly the kidney (24.1%), the central nervous system (22.6%), the liver (19.6%) and the spleen (11.3%). CONCLUSION: Contrary to published data, our results show that hydatid cysts of the kidney and of the central nervous system are more frequent than hepatic location which ranks 3rd. Such unusual results may be due to a selection bias.

[Malignant degeneration of benign cystic teratoma of the ovary with synchronous mediastinal teratoma. A case report]. / Association synchrone d'un tératome de l'ovaire cancérisé associé à un tératome du médiastin. A propos d'un cas.

Squamous cell carcinoma arising in mature cystic teratoma of the ovary is an uncommon complication occurring usually in elderly woman. We present an unusual case of a post-menopausal woman with a synchronous mediastinum teratoma. While ovary tumor was symptomatic, mediastinal teratoma was an incidental finding. To the best of our knowledge, this association is the first to be reported in literature.

Cutaneous gamma-delta T-cell lymphoma arising in the setting of Behcet's disease.

Cutaneous gamma-delta T-cell lymphoma (CGD-TCL) is an uncommon lymphoma composed of a clonal proliferation of mature activated gamma-delta T-cells expressing a cytotoxic phenotype. Malignant lymphoma is rarely associated with Behcet's disease, as only 12 cases have been reported in the literature, including a case of cutaneous T-cell lymphoma. In this report, the authors present a new case of CGD-TCL emerging in the course of Behcet's disease in a 40-year-old man. Diagnosis of CGD-TCL was established based on the combination of clinical, histological, immunophenotypical and molecular findings. Through a review of the current literature, the authors analyse the unique clinicopathological, molecular and immunohistochemical features of this rare cutaneous lymphoma.

Gynandroblastoma. Report of an unusual ovarian tumour and literature review.

Gynandroblastoma is a rare variant of ovarian sex cord stromal tumours that demonstrates morphological evidence of both male and female differentiation. We report a new case of gynandroblastoma in a 22-year-old nulliparous female with a history of menstrual disturbance. Physical examination disclosed a painless pelvic mass measuring 20 cm across with normal secondary sex characteristics, and no signs of virilisation. Histological examination of the surgically resected primary tumour revealed a predominant adult granulosa cell component admixed with a minor Sertoli cell component that did not exceed 20% of the tumour. Immunohistochemical analysis showed positive immunostaining of Sertoli cell areas with inhibin. The final diagnosis was gynandroblastoma. The post-operative course was uneventful and there was no evidence of recurrence during the 9-month follow-up period.

Renal epithelioid angiomyolipoma: a case report and literature review.

BACKGROUND: Renal epithelioid angiomyolipoma is a recently recognized variant of angiomyolipoma, closely simulating renal cell carcinoma both clinically and histologically. Only a relatively small number of cases of epithelioid angiomyolipoma of the kidney have been reported. AIM: To highlight clinicopathological features of this rare tumour. OBSERVATION: We report herein a new case of renal epithelioid angiomyolipoma in a 38-year-old male with no stigmata of tuberous sclerosis. The tumour was composed of diffuse sheets of epithelioid cells, small numbers of adipocytes and occasional blood vessels. Immunohistochemically, neoplastic cells were immunoreactive for HMB-45, but negative for cytokeratin. The patient showed no evidence of recurrence or metastatic disease one year after radical nephrectomy. CONCLUSIONS: Epithelioid angiomyolipoma may be locally aggressive and can metastasise; therefore, long-term post-operative follow-up is mandatory.

Subependymomas: a clinicopathological study of 6 symptomatic cases.

BACKGROUND: Subependymomas are rare, slow-growing, ependymal neoplasms that commonly occur in the fourth or lateral ventricles. OBJECTIVE AND DESIGN: A retrospective study of 6 histologically proven subependymomas was undertaken to analyse their clinicopathological characteristics. RESULTS: There were five male and one female patients ranging in age from 11 to 50 years (mean 35.8 years). All patients were symptomatic at diagnosis. The most common clinical presentations included headache (n=6) and vomiting (n=3). Tumours were located in the lateral ventricle in five cases and in the fourth ventricle in one case. Magnetic resonance imaging detected obstructive hydrocephalus in all cases. Five patients underwent gross total resection and one patient had subtotal excision of the tumour. Histologically, all tumours were characterised by clustering of isomorphic cells arranged against a fibrillary background. Focal cystic degeneration was seen in 5 tumours. During the follow-up period, which ranged between 2 months and 10 years, all patients were symptom-free with no evidence of recurrence.