Sporadic Zollinger-Ellison syndrome in a patient with isolated mesenteric gastrinoma.

INTRODUCTION: Zollinger-Ellison syndrome (ZES) is due to a gastrin-producing neuroendocrine tumor (gastrinoma) with subsequent gastric acid hypersecretion, gastroesophageal reflux disease (GERD), peptic ulcers, and chronic diarrhea. CASE PRESENTATION: A 40-year old patient presented with epigastric pain associated with diarrhea evolving for 10 years, he has been treated by acid-suppressive medications without substantial clinical improvement. Gastro-duodenal endoscopy was performed with gastric biopsies that showed peptic ulcerations, a serum gastrin assay that showed high levels exceeding 10 times the upper limit of the normal range. The abdominal contrast-enhanced computed tomography scan (CT-Scan) showed a solid-cystic mass with contrast enhancement in the right colon mesentery. The histopathological analysis of the resected mass showed a well-differentiated neuroendocrine tumor, and the diagnosis of a sporadic Zollinger-Ellison syndrome (ZES) was disclosed. Eighteen months after the surgical treatment the patient has no symptoms of the disease. CLINICAL DISCUSSION: Patients with ZES present often with long-standing clinical symptoms with delayed diagnosis. The surgical resection of the causative gastrinoma remains the most effective therapeutic treatment. CONCLUSION: Patients with refractory chronic diarrhea and epigastric pain should be suspected of having ZES in order to benefit from adequate clinical management.

An inflammatory myofibroblastic tumor of the ampulla of Vater, an exceptional location: a case report.

Introduction and importance: Inflammatory myofibroblastic tumors constitute a group of mesenchymal tumors associated with inflammatory infiltration. They occur mainly in young patients. It is classified by the World Health Organization as a borderline neoplasm. They are observed in many organs, particularly the lungs. Digestive localization is rare, and localization into the ampulla of Vater has been reported once. Case presentation: We report the case of a 39-year-old patient who was admitted for cholestatic jaundice with right hypochondrium pain. Computed tomography and magnetic resonance imaging revealed a tumor at the biliopancreatic junction. A cephalic duodenopancreatectomy was performed, and a histological examination of the surgical specimen revealed an inflammatory myofibroblastic tumor of the ampulla of Vater. The postoperative evolution was without any complications. Clinical discussion: This is the second case of localization of an inflammatory myofibroblastic tumor in Vater's ampulla. The therapeutic approach is the complete excision of these inflammatory tumors, thus reducing the risk of local recurrence. In the literature, all cases of incomplete excision have resulted in recurrences. Conclusion: Inflammatory myofibroblastic tumors are rare. The diagnosis was based on histopathological findings and confirmed using immunohistochemical techniques.

Appendiceal mucocele as an exceptional cause of ileocecocolic intussusception in adults: a case report.

BACKGROUND: Intussusception is a rare condition in adults, accounting for 5% of intestinal intussusception and being responsible for approximately 1% of all adult bowel obstructions. Neoplastic origin is the most common etiology of intestinal intussusception in adults, unlike pediatric intussusception, which is usually idiopathic. Intussusception due to the appendiceal mucocele is exceptional, and only a few cases have been reported in the medical literature. CASE PRESENTATION: We report the case of a 25-year-old black African male patient with no medical history. He presented to the emergency department for abdominal pain, nausea, and bilious vomiting. The abdominal examination revealed typical signs of acute bowel obstruction. Enhanced abdominopelvic computed tomography showed an invagination of the last ileal loop, cecum, and ascending colon into the lumen of the transverse colon, with a rounded image with hypodense content and some calcifications compatible with an appendiceal mucocele. An emergency exploratory laparoscopy was performed and confirmed the ileocecocolic intussusception. Right hemicolectomy and ileocolic anastomosis were performed. The patient recovery postoperatively was uneventful, and he was discharged 4 days later. Histological examination of the surgical specimen confirmed the diagnosis of mucinous cystadenoma. CONCLUSION: The symptoms of bowel intussusception with the appendiceal mucocele as the lead point in adults are similar to any other bowel intussusception. Differential diagnosis is often carried out thanks to the injected abdominal computed tomography scan.

Splenic lymphangiomas as a common indication for splenectomy: a case series with literature review.

BACKGROUND: Splenic lymphangiomas (SL) are very rare benign cystic lesions found in pediatric population. Their occurrence in adults is exceptional. Splenectomy is the common management of splenic lesions for diagnostic and/or therapeutic purpose. Our aim is to report additional cases of SL diagnosed on splenectomy specimens at our Pathology laboratory with literature review. METHODS: This is a retrospective study including all cases of splenectomy recorded at our Pathology laboratory (June 2020-August 2022). We performed a comparison of clinicopathological features between patients with SL and those with other benign splenic diseases. RESULTS: Sixteen cases of splenectomy were included. The mean age was 30.25 years (range of 6-70 years). The final histopathological diagnoses were congestive spleens in all cases of sickle cell disease (SCD) (5/16 patients, 31.25%), splenic cystic lymphangiomas (4/16 patients, 25%), capsular splenic infiltration by gastric and colic cancers (3/16 cases, 18.75%), splenic abscess (2/16 cases, 12.5%) and splenic rupture with subcapsular hematoma (1/16 patients, 6.25%). 12/16 patients (75%) had benign splenic conditions (4/12 with SL, 5/12 with SCD, 2/12 with abscess and 1/12 with splenic trauma). Patients with SL were older than those with other benign splenic conditions (mean age of 28.27 years versus 20.87 years). Also patients with SL presented with massive splenomegaly (mean splenic weight of 1675 g versus 418.75 g, mean splenic size of 19.62 cm versus 14.63 cm). Open surgery was performed in 15/16 patients (93.75%). CONCLUSION: Unlike previous studies, our series shows that SL are a common indication for splenectomy and occur in older patients with massive cystic splenomegaly. Open splenectomy is still an usual surgical practice in our country.

Urinary bladder Schistosoma haematobium-related squamous cell carcinoma: a report of two fatal cases and literature review.

BACKGROUND: Schistosomiasis is still a public health issue in certain areas of developing countries (especially in sub-saharan Africa). Schistosoma haematobium is a proven carcinogenic agent that causes mainly bladder squamous cell carcinoma. This type of cancer has characteristic epidemiological, clinical and histopathological features with poor prognosis as compared to other urinary bladder cancers not associated with this parasite. CASES PRESENTATION: We report two fatal cases of advanced-stage bladder squamous cell carcinoma associated with Schistosoma haematobium in a sub-saharan developing African country (Niger), illustrating the devastating complications of this tropical neglected disease. The two cases were a 38-year-old woman and a 37-year-old male. They presented with chronic pelvic pain and hematuria. The clinical and radiological work-up revealed invasive urivary bladder tumor extended to the pelvis, that was histopathologically proven to be an invasive squamous cell carcinoma associated with Schistosoma haematobium. The two patients died shortly after the diagnosis before chemotherapy prescription. CONCLUSION: Schistosoma-associated bladder squamous cell carcinoma has characteristic features with dismal prognosis. Eradication of this parasite remains the only efficient way to prevent the devastating consequences of this particular cancer.

Proptosis Revealing a Rare Lacrimal Gland Tumor: A Case of Chondroid Syringoma in a 35-year-old Patient.

Lacrimal gland chondroid syringoma is a very rare tumor with classic clinico-radiological symptoms that should be familiar to clinicians for appropriate patients' management as the tumor has potential for recurrence and malignant transformation. We report herein a case of chondroid syringoma in a 35-year-old patient presenting with progressive painless proptosis for 2 years. He underwent complete surgical removal of the tumor, with subsequent clinical improvement of his symptoms.

Comparative analysis of clinicopathologic features between adenoma and hyperplasia in surgically treated patients for hyperparathyroidism: A retrospective study.

BACKGROUND: Hyperparathyroidism (HPT) is a common endocrine disorder resulting from overproduction of parathyroid hormone (PTH). Usually HPT is caused by parathyroid adenoma (PA) or parathyroid hyperplasia (PH). Our aim is to assess clinicopathologic features associated with PA and PH in patients with HPT. METHODS: We retrospectively collected 29 cases of HPT recorded at the Department of Pathology of Hassan II University Hospital of Fes, Morocco, from 2013 to 2016. RESULTS: The mean age was 52.14 ± 15.7 years (range of 22-76 years), 13 patients (44.8%) had primary HPT, 16 (55.2%) had secondary HPT. The largest size of the resected parathyroid specimens ranged from 1 to 3.6 cm (mean of 2.26 ± 0.66 cm). Seventeen patients (58.6%) had PA, the remaining cases were diagnosed as PH. There were no significant statistical differences between PA and PH in age, sex, clinical presentation, preoperative serum PTH, or in parathyroid gland size (P > 0.05). However compared to PH, PA is more often a single-gland disease, found in primary HPT with higher preoperative calcium level (P ˂ 0.05). CONCLUSIONS: In patients surgically treated for HPT, PA is associated with some distinctive clinicopathologic features. These findings could be helpful to pathologists and clinicians for appropriate clinicopathologic management.

Gastric Signet Ring Cell Carcinoma: A Comparative Analysis of Clinicopathologic Features.

Signet ring cell carcinoma (SRC) is a distinct histological subtype of gastric carcinoma. Our aim is to investigate differential characteristics between gastric SRC and other non SRC carcinomas (nSRC). It was a retrospective study including 183 patients diagnosed with gastric carcinoma over a period of 5 years at our pathology department. We performed statistical comparison of clinicopathological features between patients with SRC and those with nSRC. 127 patients (69.4%) had nSRC, 56 had SRC (30.6%), the mean age was 56.67 ± 14.03 years. Patients with SRC were younger than those with nSRC (mean age of 49.66 versus 59.76, P = 0.030). Patients with SRC tend to have more diffuse tumors in the stomach (P = 0.005), with flat macroscopic appearance (P = 0.001). Patients with SRC present more often with pT3 tumors (P < 0.001), lymph node metastasis (P = 0.024) and perineural invasion (P = 0.003). There were no significant differences between SRC and nSRC in gender, vascular invasion or distant metastasis (P > 0.05). The median survival time was 42.82 ± 1.70 months. Patients with nSRC live longer than those with SRC, but the difference was not significant (P = 0.28). SRC is a histological subtype of gastric carcinoma with distinctive clinicopathologic features. The clinical management of patients should take into account these particular features.

The value of intraoperative parathyroid hormone assay in the surgery of mediastinal ectopic parathyroid adenoma (A case series).

BACKGROUND AND AIMS: Ectopic parathyroid adenoma is a rare entity. Its clinical management is challenging due to varying locations in the mediastinum. The aim of our study is to report our experience about the preoperative localization of the ectopic parathyroid adenoma and to emphasize the major role of the intraoperative parathyroid hormone assay in such circumstances. METHODS: It is a monocentric, retrospective study about patients diagnosed with EPA (ectopic parathyroid adenoma) from January 2015 to December 2016. Clinical aspects, preoperative management as well as the surgical procedures have been analyzed. RESULTS: There were 7 women, with an average age of 59.14 years. Six patients presented with biological disorders of the phosphocalcic metabolism such as spontaneous bone fracture and recurrent renal lithiasis. In one case, EPA was discovered in the setting of malignant hypercalcemia. The topographic preoperative assessment with a cervicothoracic CT (computed tomography) showed spontaneously hyperdense tissular masses of variable localizations in the mediastinum. A Tc-99 m (99mTc - MIBI) scintigraphy was performed in 5 patients and showed uptake in all cases. We performed cervicotomy in 1 case, manubriotomy in 2 patients, neck manubriotomy in 2 cases, total vertical sternotomy in 1 case, and posterolateral thoracotomy in 1 patient. The lesion was localized in the mediastinum in 1 patient in the perithymic fat in 1 case; EPA was laterotracheal in 1 case, retro tracheal in 1 case, intra-thymic in 2 cases, inter-jugulo-carotidian with contact with the left subclavial artery in 1 case, and anterior mediastinal in 1 patient. The 1-h after-parathormonemia following removal of the surgical specimens showed a decrease value of 45 and 80% of the baseline value. No surgical morbidity was noted after an average follow-up of 7.9 months (range of 5-18 months). CONCLUSION: The preoperative topographic diagnosis of ectopic parathyroid adenoma is challenging for the surgeon despite progress in the morphological assessment. The intraoperative parathyroid hormone assay is a valuable tool for an appropriate surgical management.

How thoracic surgeon manage tracheal tumors in African country? (Case series).

INTRODUCTION: Tracheal tumors are a rare pathological entity whose diagnosis is usually delayed by clinical latency. Surgery, which consists of a tracheal resection-anastomosis with or without reconstructive reconstruction, remains the treatment that ensures the best long-term survival. METHODS: This is a retrospective study about 8 patients admitted in the department of thoracic surgery of Hassan II's university hospital of Fes for tracheal tumors management during 7 years time (December 2010 to December 2017). RESULTS: There were 6 men and 2 women with an average age of 44.4 years ranged from 17 to 65 years, 4 were smokers. Dyspnea was the main trigger sign. Seven (7) have undergone bronchial fibroscopy diagnostic with a finding of budding process in 5 patients, the middle of the trachea is often concerned in 3 patients, obstructing the lumen of the trachea in almost all patients. The treatment in all patients was surgical with an intubation via the operative field, 4 trachea resection-anastomosis, 4 plasty (Lateral resection with V plasty, Kergin's plasty, Mattey's tracheobronchial anastomosis and widened V-resection to the carina). The most common histological type in our series was Adenoid Cystic Carcinoma in 2 of our patients. For the other patients we have found squamous cell carcinoma (1 case), adenocarcinoma (1 case), atypical carcinoid tumor (1 case), low grade mucoepidermoid carcinoma (1 case), an adenoma pleomorph (1 case) and endotracheal goiter (1 case). The operative follow-up was simple in 7 of our patients, all of whom underwent post-operative fibroscopy within an average of 9 days. Two cases of post-operative recurrent palsy had been observed, all of which had progressed well under treatment. We have noted 2 deaths, including one at day 4 post-operative, and the other died from complications of post-radiation tracheal stenosis. Back to 32 months' average follow-up, we have enregistered a case of a distant relapse by cervical lymph node metastasis in one patient, 5 years after surgery. CONCLUSION: Primary tumors of the trachea remain of reserved prognosis with 5-year survival of 57% of all histological types combined. Computed tomodensitometry and tracheobronchial fibroscopy remain the means of reference exploration in the diagnosis and assessment of surgical resectability.

Unusual mucosal lesion: A case of rectal pseudolipomatosis in a 60-year-old patient.

Pseudolipomatosis is a very rare benign pathologic condition of colorectal mucosa. It is caused by the presence of gas bubbles in the intestinal mucosa. The endoscopic and histologic aspects are misleading as they suggest adipocytic or vascular lesions. We present herein a case of rectal pseudolipomatosis in a 60-year-old woman presenting with rectal bleeding. The endoscopic pattern was not suggestive of the lesion, and the histopathologic analysis of the patient's rectal biopsies revealed characteristic features of pseudolipomatosis. Rectocolic pseudolipomatosis is a very rare benign condition with challenging clinicopathologic presentation. Clinicians and pathologists should be aware of this uncommon lesion for correct diagnosis and appropriate clinical management.

Female genital tuberculosis: a clinicopathological report of 13 cases.

Female genital tuberculosis (FGTB) is a rare form of tuberculosis (TB) affecting women of reproductive age. The clinical presentation is often misleading. We retrospectively collected cases of female genital tuberculosis (FGTB) diagnosed from 2006 to 2016 at our pathology department. The mean age was 39.92 years (range of 18-74 years). Most patients had reproductive age, with 30.76% postmenopausal women. Seven cases (53.84%) were addressed for histopathological analysis for clinical malignant suspicion. The diagnosis was made on eight surgical specimens (61.53%). Nine patients (69.23%) had multifocal TB. The most involved genital organs were fallopian tubes (63.84%), followed by ovaries (46.15%), endometrium (38.46%) and the cervix (23.07%). The macroscopic aspects of all resected specimens did not show the classic appearance of caseous necrosis. We found that most patients with genital tuberculosis present with clinical suspicion of malignancy leading to aggressive surgical management.

Langerhans cell histiocytosis presenting as eosinophilic granuloma of the bilateral forearms in an 8-year-old girl: a case report.

BACKGROUND: Langerhans cell histiocytosis previously known as histiocytosis X is a rare disease of children and young adults with a very broad clinical spectrum. In children, its annual incidence is estimated between 0.2-0.5 per 100,000. CASE REPRESENTATION: An 8-year-old Moroccan girl with no known personal or family history presented to our institution with painful swelling of both forearms. An X-ray and magnetic resonance imaging were inconclusive. We then performed a biopsy curettage (of her left forearm). Microscopic analysis followed by immunohistochemical analysis disclosed a diagnosis of Langerhans cell histiocytosis. No chemotherapy was necessary. Clinical and radiological improvement was achieved after 6 months. CONCLUSION: The particularity of this observation is the bilaterality of the lesion on both forearms and it has not previously been reported. Langerhans cell histiocytosis should be included in the differential diagnosis of osteomyelitis and Ewing's sarcoma.

Extra-uterine low grade endometrioid stromal sarcoma arising from ovarian endometriosis: a case report and review of the literature.

BACKGROUND: Endometrial stromal sarcoma (ESS) is a rare neoplasm accounting for only 0.2% of female genital tract tumors. The primary extra-uterine location of ESS is an extremely uncommon occurrence. CASE PRESENTATION: We present a case of a 64-year-old woman presenting with abdominopelvic and bilateral ovarian tumors with misleading clinical presentation and diagnostic challenge. The histopathological examination of the resected specimens disclosed the diagnosis of primary extra-uterine ESS arising from ovarian endometriosis. Adjuvant therapy with an aromatase inhibitor drug was prescribed for the patient, and she is still alive with no evidence of disease 7 months after surgery. CONCLUSION: The awareness of the potential extra-uterine location of ESS should lead to correct diagnosis as this tumor has histopathological features and clinical behavior similar to its uterine counterpart.

Sclerosing angiomatoid nodular transformation of the spleen (SANT) in a patient with clear cell carcinoma of the uterus: a case report.

BACKGROUND: Sclerosing angiomatoid nodular transformation of the spleen is a very rare benign vascular lesion recently described. Usually, sclerosing angiomatoid nodular transformation of the spleen is an incidental finding; the association with malignant tumors is extremely rare. To the best of our knowledge, we report the first case of sclerosing angiomatoid nodular transformation of the spleen associated with uterine clear cell carcinoma. CASE PRESENTATION: A 49-year-old Arabic woman presented to our institute with abdominal pain and distention. An abdominal computed tomographic scan was obtained, which showed a 14-cm uterine malignant tumor and a 4-cm isolated splenic nodule suggesting a metastatic lesion. The tumor was limited to the uterus but did not extend beyond. The patient underwent surgical treatment, and the histopathological examination of the resected uterine and splenic specimens disclosed invasive uterine clear cell carcinoma and sclerosing angiomatoid nodular transformation of the spleen, respectively. The patient had no signs of the disease 17 months after surgical treatment. CONCLUSIONS: Sclerosing angiomatoid nodular transformation of the spleen is a very rare benign disease with a misleading presentation when associated with a malignant tumor. Pathological assessment of the resected spleen is the only way to achieve the correct diagnosis.

An Unusual Mass of Posterior Mediastinum: A Case of Retrotracheal Parathyroid Adenoma Presenting With Primary Hyperparathyroidism.

Although parathyroid ectopy in the mediastinum has been the subject of several publications, its location in the posterior mediastinum is very rarely reported. We report a case of a 69-year-old patient who presented with clinical symptoms of malignant hypercalcemia due to a retrotracheal mediastinal parathyroid adenoma. The surgical excision leads to a quick normalisation of the phosphocalcic balance with improvement of the clinical symptoms. Ectopic hypersecreting parathyroid adenoma with life-threatening hypercalcemia should prompt radiological assessment and appropriate surgical management to prevent further clinical complications.

The Assessment of HER2 Gene Status by Fluorescence In Situ Hybridization in Invasive Breast Carcinomas With Equivocal HER2 Immunostaining: Experience From a Single Institution.

BACKGROUND: A subset of breast carcinomas harbors overexpression of the human epidermal growth factor receptor 2 (HER2). Fluorescence in situ hybridization (FISH) should be performed in breast carcinomas with equivocal HER2 immunostaining (immunohistochemistry [IHC] HER2 2+). The aim of our study is to investigate clinicopathologic factors associated with HER2 status in breast invasive carcinomas with IHC HER2 2+ through FISH analysis. METHODS: This is a retrospective study including the FISH analysis of 111 patients with invasive breast carcinomas with equivocal HER2 immunostaining. RESULTS: The mean age was 49.51 ± 10.48 years, and invasive breast carcinoma of no special type was the most histological type in our study (96.4%). Most patients had tumors positive for hormones receptors (88.2% positive for estrogen receptor and 81.4% for progesterone receptor). On FISH, the HER2 amplification rate was 22.5%. There was no significant association of HER2 status with any clinicopathologic factors ( P > .05). CONCLUSIONS: Our study shows that there are no reliable clinicopathologic factors to predict the HER2 status in breast tumors with equivocal HER2 immunostaining, supporting the necessary usage of FISH in such circumstances.

Isolated splenic lymphangioma presenting as a huge mass causing anemia and abdominal distension in an adult patient: a case report.

BACKGROUND: Lymphangiomas are uncommon benign lesions of lymphatic vessels very rarely affecting the spleen. Isolated involvement of the spleen in adult patients is rarely reported. CASE PRESENTATION: We report a case of a 40-year-old Arabic woman who presented with a 25-cm abdominal mass, fatigue, and anemia evolving for 6 months. Her physical examination revealed anemic syndrome and an enormous splenomegaly extending beyond the umbilical area. An abdominal computed tomographic scan showed a 25-cm splenic mass with multiple hypodense nodules without enhancement after contrast injection. A surgical total splenectomy was performed. Histopathological analysis led to the diagnosis of cystic splenic lymphangioma. The patient's postoperative course was uneventful, and she was discharged from the hospital. CONCLUSIONS: Isolated splenic lymphangioma in adult patients is very rare. The preoperative diagnosis is challenging because imaging techniques are not specific. Pathological analysis of the resected specimen is the only effective way to render the definitive diagnosis. Splenic lymphangiomas have a benign course after complete surgical resection.

Tubular Adenoma of the Breast: A Clinicopathologic Study of a Series of 9 Cases.

Tubular adenoma of the breast is one of the most rare benign neoplasms, accounting for only 0.13% to 1.7% of all breast benign tumors. Little is known about this rare neoplasm as the current literature offers only some case reports or a few number of small series. The aim of our study is to provide some clinicopathologic features of the breast tubular adenoma. We retrospectively analyzed at our department of pathology all cases of breast tubular adenomas confirmed by immunohistochemistry over a period of 9 years (2009-2017). Nine cases of breast tubular adenoma have been recorded, with an average age of 31.44 years. Five tumors were located at the right side (55.55%), and most cases had suspicious aspects on imaging techniques (6 cases out of 9). The diagnosis has been made on 5 resected specimens (lumpectomy) and on 4 core needle biopsies. The tumor size ranged from 0.9 to 7 cm (mean size of 3.08 cm) and had well-circumscribed margins with elastic consistency. The histopathologic analysis showed a typical pattern of proliferating round and uniform tubules lined by regular epithelial cells surrounded by myoepithelial cells, packed in a small amount of stroma, highlighted by CD34 immunostaining. Tubular adenoma is a rare breast benign neoplasm of young premenopausal women. The radiologic aspects are often worrisome and only the histopathologic analysis can achieve the correct definitive diagnosis by excluding all potential differential diagnoses.