Mayer-Rokitansky-Kuster-Hauser syndrome and ovarian benign teratoma: a case report

Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH) is an unknown congenital etiology disorder characterized by agenesia or hypoplasia of the Müller ductal system, including the upper vagina, uterus and fallopian tubes. The occurrence of an associated ovarian tumor is rare, with fewer than 20 cases reported to date according to the literature. We report the case of a 14-year-old girl, virgin, who had not yet seen her menarche, complaining of an abdomino-pelvic mass associated with pain. The ultrasound performed revealed a large left ovarian tumor and an absence of uterus. The indication of a laparotomy confirmed the ovarian mass and a complete absence of uterus associated with vaginal hypoplasia. The contralateral ovary was present, and of normal appearance. The pathological examination was in favor of a mature benign multi-tissular teratoma. This is the first case described in our service. The mode of transmission of this entity appears to be autosomal dominant with low penetrance and variable expressivity, suggesting that the incidence of this syndrome is likely underestimated. With the development of techniques of medical assistance to procreation, maternity remains possible, particularly through gestational surrogacy.

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A rare presentation of benign Brenner tumor of ovary: a case report

International Journal of Reproduction Contraception Obstetrics and Gynecology ◽

10.18203/2320-1770.ijrcog20182920 ◽

2018 ◽

Vol 7 (7) ◽

pp. 2971

Author(s):

Sumathi Periasamy ◽

Subha Sivagami Sengodan ◽

Devipriya . ◽

Anbarasi Pandian

Keyword(s):

Abdominal Pain ◽

Ovarian Tumor ◽

Ovarian Neoplasms ◽

Pelvic Mass ◽

Ovarian Tumors ◽

Pathological Examination ◽

Rare Presentation ◽

Brenner Tumor ◽

Benign Nature ◽

Brenner Tumors

Brenner tumors are rare ovarian tumors accounting for 2-3% of all ovarian neoplasms and about 2% of these tumors are borderline (proliferating) or malignant. These tumors are commonly seen in 4th-8th decades of life with a peak in late 40s and early 50s. Benign Brenner tumors are usually small, <2cm in diameter and often detected incidentally during surgery or on pathological examination. Authors report a case of a large, calcified benign Brenner tumor in a 55-year-old postmenopausal woman who presented with complaint of abdominal pain and mass in abdomen. Imaging revealed large complex solid cystic pelvic mass -peritoneal fibrosarcoma. She underwent laparotomy which revealed huge Brenner tumor weighing 9kg arising from left uterine cornual end extending up to epigastric region. Myomectomy and hysterectomy with bilateral salphingo oophorectomy, omentectomy done and the specimen was sent for histopathological and immunohistochemistry study which revealed benign Brenner tumor. Brenner tumors are rare ovarian neoplasms accounting for 2-3% of all ovarian tumors. Benign Brenner tumors are usually small and solid whereas borderline and malignant Brenner tumors are usually larger and cystic with solid areas. But it is possible to have a completely benign large Brenner tumor. Therefore, benign nature of the lesion should not be excluded even when the ovarian tumor is very large.

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Demons meigs’ syndrome secondary to begnin brenner tumor with high ca125 plasmatic level: first case described in kara teaching hospital

10.36811/ojgor.2019.110002 ◽

2019 ◽

pp. 08-12

Author(s):

Aboubakari AS ◽

Dossouvi T ◽

Logbo-Akey KE ◽

Ajavon DR ◽

Dagbé M ◽

...

Keyword(s):

Pleural Effusion ◽

Teaching Hospital ◽

Ovarian Tumor ◽

Pelvic Mass ◽

Abdominal Distension ◽

Abdominal Ultrasound ◽

First Case ◽

Brenner Tumor ◽

Benign Ovarian Tumor ◽

Plasmatic Level

To report the first case of Demons-Meigs’ syndrome secondary to benign Brenner tumor with high CA125 plasmatic level managed in kara teaching hospital. A 40-year-old female patient was admitted with a 6-month history of abdominal distension. Clinical examination found abdomino-pelvic mass and declive dullness. Abdominal ultrasound found heterogeneous abdomino-pelvic mass of 180 mm in length and a large free ascites. Pelvic computorized tomography scan found heterogeneous extra-uterine mass of 180 mm in length, large ascites without pelvic or lombo-aortic lymphadenopathy or tumoral extension. Front thorax X-ray found bilateral pleural effusion; CA125 plasmatic level was 1138 IU/ml. Median laparotomy allow to aspire 2 liters of ascitic fluid and to perform left adnexectomy. Histological examination of surgery sample diagnosed begnin ovary Brenner tumor. Outcome after surgery was without complications with disappearance of ascites and pleural effusion, and CA125 plasmatic level back to normal value. Demons-Meigs’ syndrome is a rare benign ovarian tumor whose symptoms looks like ovarian cancer. Its fundamental characteristic is the disappearance of symptoms after ovarian tumor removal.

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Pyosalpinx due to Cronobacter sakazakii in an elderly woman

BMC Women s Health ◽

10.1186/s12905-021-01283-8 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Satoshi Ohira ◽

Eri Ikeda ◽

Kyosuke Kamijo ◽

Tomokuni Nagai ◽

Koji Tsunemi ◽

...

Keyword(s):

Abdominal Cavity ◽

Lower Abdominal Pain ◽

The Elderly ◽

Pathological Examination ◽

Milk Formula ◽

Adult Women ◽

Cronobacter Sakazakii ◽

Left Edge ◽

Powdered Milk ◽

First Case

Abstract Background Cronobacter sakazakii (C. sakazakii) is a bacterium known to cause severe neonatal infections in premature infants with the consumption of contaminated powdered milk formula. Adult infections are rare, and there have been no reports of pyosalpinx due to C. sakazakii to date. Case presentation We report a case of left pyosalpinx due to C. sakazakii in a sexually inactive postmenopausal woman. A 70-year-old woman presented to our hospital with left lower abdominal pain and fever. Abdominal computed tomography disclosed a cystic mass continuous with the left edge of the uterus. Urgent laparotomy revealed a ruptured left pyosalpinx with pus-like content. Left salpingo-oophorectomy, resection of the right tube, and washing of the abdominal cavity with saline were performed. Pathological examination of the left adnexa showed tubal tissue with acute inflammation and inflammatory exudate, which were compatible with pyosalpinx, and pus culture yielded C. sakazakii. Conclusions This is the first case report of pyosalpinx due to C. sakazakii. Cronobacter sakazakii infections in adult women might occur in the elderly, whose immunity has weakened. Further accumulation of cases of C. sakazakii infection is needed to clarify the etiology and behavior of C. sakazakii in adults.

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Management of Type 2B von Willebrand Disease during Pregnancy

Acta Haematologica ◽

10.1159/000453389 ◽

2017 ◽

Vol 137 (2) ◽

pp. 89-92 ◽

Cited By ~ 2

Author(s):

David McLaughlin ◽

Ron Kerr

Keyword(s):

Autosomal Dominant ◽

Case Reports ◽

Management Plan ◽

Von Willebrand Disease ◽

Best Management ◽

First Case ◽

Von Willebrand ◽

Willebrand Factor ◽

Ristocetin Cofactor ◽

Platelet Transfusions

Type 2B von Willebrand disease is a rare bleeding condition resulting in thrombocytopenia and a reduction in large VWF multimers. It usually has an autosomal dominant pattern of inheritance. We report the management of a patient with type 2B von Willebrand disease, whose diagnosis was confirmed by demonstration of a R1306W mutation, through her first pregnancy. The patient's von Willebrand factor (VWF) antigen and VWF ristocetin cofactor levels rose throughout pregnancy, with an associated drop in the platelet count. The patient was successfully managed through labour to a surgical delivery with VWF concentrate, platelet transfusions and tranexamic acid. The patient delivered a male baby who was found to have inherited type 2B von Willebrand disease and had a significant cephalhaematoma at delivery. The baby was managed with VWF concentrate and platelet transfusions and made a full recovery. There is a lack of evidence to guide the best management of pregnant patients with type 2B von Willebrand disease. We adopted a pragmatic management plan, in keeping with other published case reports. To the best of our knowledge, this is the first case report in which the child was found to have inherited type 2B von Willebrand disease and encountered bleeding problems, making this case unique amongst the published literature.

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A rare benign genitourinary tumor in a Japanese male: urinary retention owing to aggressive angiomyxoma of the prostate

Rare Tumors ◽

10.4081/rt.2010.e15 ◽

2010 ◽

Vol 2 (1) ◽

pp. 43-45 ◽

Cited By ~ 1

Author(s):

Yugo Sawada ◽

Fumio Ito ◽

Hayakazu Nakazawa ◽

Nobuhiko Tsushima ◽

Hikaru Tomoe ◽

...

Keyword(s):

Renal Dysfunction ◽

Urinary Retention ◽

Bladder Neck ◽

English Literature ◽

Pathological Examination ◽

Aggressive Angiomyxoma ◽

Urethral Meatus ◽

First Case ◽

Japanese Male ◽

Old Japanese

Close examination of a 67-year-old Japanese man, who complained of persistent nocturia, revealed that a semitransparent polypoid tumor had developed from the bladder neck to the prostatic urethra obstructing the internal urethral meatus, which resulted in excessive urinary retention and post-renal dysfunction. The tumor was resected by a transurethral procedure and a pathological examination of specimens revealed aggressive angiomyxoma (AAM) of the prostate. AAM usually develops in the intrapelvic and perineal organs of females. So far as we know, this is the second case of primary prostatic AAM reported in the English literature, and is the first case where the patient encountered urethral obstruction.

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Giant jejunal gasto-intestinal stromal tumor: A rare cause of abdominal mass in an elderly female

African Journal of Oncology ◽

10.54266/ajo.1.2.65.xvep9876 ◽

2021 ◽

Vol 1 (2) ◽

pp. 65-67

Author(s):

AC Diallo ◽

A Ndong ◽

I Niang ◽

MB Ba ◽

JA Thiam ◽

...

Keyword(s):

Abdominal Mass ◽

Pelvic Mass ◽

Final Diagnosis ◽

Progressive Increase ◽

Stromal Tumor ◽

Biological Assessment ◽

Pathological Examination ◽

Tissue Mass ◽

Surgical Specimen ◽

One Year

OBJECTIVE: We report the case of a patient presenting an abdominal mass whose final diagnosis is a gastrointestinal stromal tumor (GIST). CLINICAL OBSERVATION: It was a 61-year-old patient with no pathological history received for a progressive increase in the volume of the abdomen evolving for one year and painless. On examination, the patient was in good general condition, with normal patterns. There was an abdomino-pelvic mass of about 20 cm long axis, mobile. The rest of the exam was unremarkable. The biological assessment was normal. Abdominopelvic computed tomography revealed a tissue mass with necrosis. During the surgical exploration, a mass developed on the mesenteric border 45 cm from the Treitz angle was noted. It is not associated with ascites or peritoneal carcinoma. Bowel resection removing the mass was performed followed by end-to-end anastomosis. Pathological examination of the surgical specimen diagnosed GIST. The postoperative course at three months was excellent both clinically and radiologically. CONCLUSION: The case that we report underlines the possible jejunal localization of GIST and the clinical presentation may be usual. It also shows the difficulty of obtaining a preoperative diagnosis and the central role of surgery in management.

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LARGE BENIGN OVARIAN TUMOR MIMICKING LATE STAGE MALIGNANT OVARIAN TUMOR

International Journal of Advanced Research ◽

10.21474/ijar01/12524 ◽

2021 ◽

Vol 9 (02) ◽

pp. 848-852

Author(s):

M. Bendahhou Idrissi ◽

◽

K. Aboulfath ◽

S. Errarhay ◽

N. Mamouni ◽

...

Keyword(s):

Clinical Examination ◽

Ovarian Tumor ◽

Benign Neoplasm ◽

Exploratory Laparotomy ◽

Point Of View ◽

Pathological Examination ◽

Multiple Biopsies ◽

Benign Ovarian Tumor ◽

Peritoneal Effusion ◽

Fear Of Cancer

Ovarian fibrothecoma is a rare, benign neoplasm of the sex cords and stroma of the ovary. Patients may present with abdominal pelvic pain and / or distension, and sometimes postmenopausal bleeding. Large tumors (> 10cm) are often associated with pleural and peritoneal effusion mimicking an advanced ovarian malignancy (Deimon â€“Meigs syndrome). We report 2 cases of giant fibrothecoma associated with ascites and pleurisy. Both patients were postmenopausal. The ovarian tumor was discovered during an increase in abdominal volume in the first patient and as part of the etiological workup of encysted pleurisy in the second. The clinical examination made it possible to objectify a tumor with abdominopelvic development of size greater than 10 cm, the clinical examination also noted, in the two patients, a right basithoracic dullness in favor of a pleurisy and an ascites of average abundance suggesting in the first place a malignant tumor of the ovary, with peritoneal carcinoma. the two patients underwent a pelvic ultrasound, supplemented by an abdominal pelvic CT scan objectifying the presence of a large ovarian mass with pleural and peritoneal effusions. Exploratory laparotomy was required in both patients and underwent an extemporaneous examination which was consistent with the definitive pathological examination in both patients. Both patients underwent total hysterectomy with bilateral adnexectomy and omentectomy with multiple biopsies as the clinical picture was highly suggestive of malignancy. The postoperative follow-up was simple with total regression of peritoneal and pleural effusions in the two cases of Demons-Meigs syndrome. The definitive pathological examination revealed a fibrothecoma. Fibrothecal tumors remain a real challenge from a diagnostic and therapeutic point of view. The fear of cancer is even greater in the presence of Demons-Meigs syndrome. The management of these tumors requires a good clinical and radiological analysis, and it is the histological examination that confirms the diagnosis. The surgery corresponds, in post-menopausal patients, to a hysterectomy with bilateral adnexectomy, while conservative treatment by unilateral adnexectomy is entirely justified in young women who still want fertility.

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Prostatic Adenocarcinoma Metastatic to Pleomorphic Liposarcoma, a “Collision Phenomenon”: Report of a Case with Review of Pelvic Collision Tumors

Pathology Research International ◽

10.4061/2011/173541 ◽

2011 ◽

Vol 2011 ◽

pp. 1-7

Author(s):

Somak Roy ◽

Ronald L. Hrebinko ◽

Kathleen M. Cieply ◽

Anil V. Parwani ◽

Uma N. M. Rao

Keyword(s):

Pelvic Mass ◽

Collision Tumor ◽

The Body ◽

Pathological Examination ◽

Prior History ◽

Pelvic Cavity ◽

Collision Tumors ◽

History Of ◽

Conclusive Diagnosis

“Collision tumor” is an uncommon phenomenon characterized by coexistence of two completely distinct and independent tumors at the same site. Collision tumors have been reported in different sites in the body; however, these are particularly uncommon in the pelvic cavity. A 70-year-old man, with prior history of urothelial and prostate cancer, presented with a large pelvic mass detected on imaging studies. Pathological examination revealed a large liposarcoma with prostatic carcinoma embedded in it. Immunohistochemistry and florescence in situ hybridization studies were performed to reach to a conclusive diagnosis. To the best of our knowledge, this is the second case reported till date. We present the challenges encountered in the diagnosis of this case and review of pelvic collision tumors.

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ALK-positive Histiocytosis of Umbilicus Subcutaneous with KIF5B-ALK Fusion: a Case Report

10.21203/rs.3.rs-147658/v1 ◽

2021 ◽

Author(s):

Yili Zhu ◽

Jun Fan ◽

Bo Huang ◽

Ying Wu ◽

Heshui Shi ◽

...

Keyword(s):

Pathological Examination ◽

Older Children ◽

Anaplastic Lymphoma ◽

Molecular Tests ◽

Spindle Cells ◽

First Case ◽

Alk Positive ◽

Painless Mass ◽

Lymphocytic Infiltrates

Abstract Background: Since the discovery of the first case of Anaplastic lymphoma kinase (ALK) -positive histiocytosis in 2008, originally described as a systemic, self-limiting disease in infants, the range of ALK-positive histiocytosis has recently been expanded to include localized diseases in older children and young adults. Case presentation: We present the case of an 18-year-old female with periumbilical painless mass for 5 months, who underwent a resection of the mass. Pathological examination showed the tumor consists predominantly of fascicular to storiform growth of nonatypical spindle cells, admixed with lymphocytic infiltrates. The tumor spindle cells were diffusely positive for CD68, CD163 and ALK. Further, molecular tests revealed ALK gene fusion: Kinesin Family Member 5B (KIF5B) (E24)-ALK (E20), confirmed ALK-positive histiocytosis. The tumor has not recurred one and a half years after resection by follow-up examination.Conclusion: ALK-positive histiocytosis in local lesion can achieve remission by complete resection and clinical follow-up showed a favorable prognosis.

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Complete resolution of a giant multilocular prostatic cystadenoma following androgen deprivation therapy: an illustrative case report

Oxford Medical Case Reports ◽

10.1093/omcr/omab053 ◽

2021 ◽

Vol 2021 (7) ◽

Author(s):

Nikita Sushentsev ◽

Yvonne Rimmer ◽

Tristan Barrett

Keyword(s):

Androgen Deprivation Therapy ◽

Complete Resolution ◽

External Beam Radiotherapy ◽

Pelvic Mass ◽

Androgen Deprivation ◽

Illustrative Case ◽

Imaging Features ◽

First Case ◽

Complete Surgical Resection ◽

Risk Prostate Cancer

ABSTRACT Giant multilocular prostatic cystadenoma (GMPC) is a rare benign pelvic mass for which complete surgical resection is an accepted treatment of choice. This report presents the first case of complete resolution of GMPC following a 3-year course of luteinising hormone-releasing hormone agonist alongside external beam radiotherapy for the concurrent treatment of unfavorable intermediate-risk prostate cancer. In addition to illustrating the imaging features of the effect of androgen deprivation therapy (ADT) and radiotherapy on GMPC regression, this case provides evidence for considering ADT as an alternative, noninvasive GMPC treatment option in patients in whom surgical treatment is either contraindicated or can be made less invasive by reducing the size of GMPC prior to its removal.

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