Primary Intestinal-type Adenocarcinoma of the Vulva Causing Secondary Extramammary Paget Disease

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S26-S26
Author(s):  
D J Forsythe ◽  
P Weisman
Keyword(s):  
Case Reports ◽  
Intestinal Type ◽  
Tumor Type ◽  
Invasive Adenocarcinoma ◽  
Squamous Mucosa ◽  
Paget Disease ◽  
Routine Surveillance ◽  
Signet Ring ◽  
History Of ◽  
Intestinal Type Adenocarcinoma

Abstract Casestudy A 65-year-old woman with a known history of anogenital extramammary Paget disease presented with a pedunculated lesion arising from the vulvar vestibule near the urethral meatus, discovered during a routine surveillance exam. Results Biopsy of the lesion revealed an adenocarcinoma with villoglandular architecture and pseudostratified columnar epithelium with goblet cells that is arising in direct continuity with the uninvolved squamous mucosa. In addition to the villoglandular component, an area of dyscohesive cells with signet ring morphology within a mucinous background was present. Biopsy of a lesion inferior to the index mass showed Paget disease with similar morphology to the invasive adenocarcinoma. By immunohistochemistry (IHC), the invasive adenocarcinoma was diffusely positive for CK20, CK7, and CDX-2 with patchy staining for p16. Metastasis was excluded by colonoscopy, mammography, and PET-CT. Additional IHC stains for GCDFP-15, GATA-3, PAX-8, S100, p63, and PSA were all negative on subsequent biopsies performed for mapping the extent of disease. These features supported a diagnosis of primary intestinal-type adenocarcinoma of the vulva and pointed to this tumor as a source of her Paget disease. Conclusion Primary intestinal-type adenocarcinomas of the vulva are extremely rare with only a small number of case reports in the literature. It is suggested that these tumors acquire a colorectal phenotype due to the cloacal origin of the vulvar vestibule. No standard treatment approach has been developed due to the rarity of this disease, but the majority of reported cases have had an indolent course. To the best of our knowledge, other than this case, there is only a single additional case report in the literature of secondary Paget disease arising from this tumor type. We underscore the propensity of this tumor to cause secondary Paget disease, mimicking spread from a colorectal primary site.

Primary vaginal adenocarcinoma of intestinal type arising from a tubulovillous adenoma

2006 ◽  
Vol 16 (3) ◽  
pp. 1461-1465 ◽  
Author(s):  
W. A.A. Tjalma ◽  
C. G.A. Colpaert
Keyword(s):  
Clinical Examination ◽  
Villous Adenoma ◽  
Present Report ◽  
Vaginal Wall ◽  
Intestinal Type ◽  
Tumor Type ◽  
Positron Emission ◽  
Appropriate Treatment ◽  
History Of ◽  
Magnetic Resonance Imaging Mri

Enteric or intestinal-type neoplasms of the vagina are extremely rare. The present report describes a 55-year-old woman who presented with a 6-week history of postmenopausal bleeding. On clinical examination, a lesion on the posterior vaginal wall was noticed. Rectovaginal examination suggested a tumor in the rectovaginal septum. Biopsy revealed an adenocarcinoma of the intestinal type, with a small remnant of a villous adenoma. The histologic interpretation pointed in the direction of a metastatic gastrointestinal tumor; yet, clinical examination, rectoscopy, colonoscopy, magnetic resonance imaging (MRI) of the abdomen, and positron emission tomography (PET) scanning excluded this possibility. This led to the conclusion that the lesion was a primary intestinal-type adenocarcinoma of the vagina that had arisen from a vaginal villous adenoma. It is important to be aware of this tumor type and to distinguish them from metastatic colorectal adenocarcinoma in order to plan appropriate treatment.

Herpetic Whitlow of the Hand in Infants

2020 ◽  
Author(s):  
Mohammad M. Al-Qattan ◽  
Nada G. AlQadri ◽  
Ghada AlHayaza
Keyword(s):  
Bacterial Infection ◽  
Case Reports ◽  
Case Series ◽  
Individual Case ◽  
Senior Author ◽  
Secondary Bacterial Infection ◽  
Clinical Appearance ◽  
History Of ◽  
Mode Of Transmission ◽  
Time Of Presentation

Abstract Introduction Herpetic whitlows in infants are rare. Previous authors only reported individual case reports. We present a case series of six infants. Materials and Methods This is a retrospective study of six cases of herpetic whitlows in infants seen by the senior author (MMA) over the past 23 years (1995–2017 inclusive). The following data were collected: age, sex, digit involved in the hand, mode of transmission, time of presentation to the author, clinical appearance, presence of secondary bacterial infection, presence of other lesions outside the hand, method of diagnosis, treatment, and outcome. Results All six infants initially presented with classic multiple vesicles of the digital pulp. In all cases, there was a history of active herpes labialis in the mother. Incision and drainage or deroofing of the vesicles (for diagnostic purposes) resulted in secondary bacterial infection. Conclusion The current report is the first series in the literature on herpetic whitlows in infants. We stress on the mode of transmission (from the mother) and establishing the diagnosis clinically. In these cases, no need for obtaining viral cultures or polymerase chain reaction; and no medications are required. Once the vesicles are disrupted, secondary bacterial infection is frequent and a combination of oral acyclovir and intravenous antibiotics will be required.

scholarly journals Spontaneous haemorrhage of an adrenal angiomyolipoma: case report

2019 ◽  
Vol 25 (1) ◽  
Author(s):  
Danielle Whiting ◽  
Ian Rudd ◽  
Amit Goel ◽  
Seshadri Sriprasad ◽  
Sanjeev Madaan
Keyword(s):  
Case Reports ◽  
Case Presentation ◽  
Open Adrenalectomy ◽  
Large Size ◽  
History Of ◽  
Loin Pain ◽  
Benign Tumours ◽  
Spontaneous Haemorrhage ◽  
Mesenchymal Tumours

Abstract Background Angiomyolipomas are rare mesenchymal tumours arising from the perivascular epithelioid cells consisting of variable amounts of adipose, thick-walled blood vessels and smooth muscle cells. These benign tumours commonly occur in the kidney with only a few case reports of adrenal angiomyolipomas which have the potential to reach a large size and haemorrhage. Case presentation A 45-year-old lady presented with a 3-week history of right loin pain, nausea and vomiting. A CT scan revealed a right adrenal angiomyolipoma measuring 6.3 × 6.8 cm with associated haemorrhage. The lesion was successfully treated with right open adrenalectomy, and histology confirmed the diagnosis of adrenal angiomyolipoma. The patient remained well with no evidence of recurrence at the 36-month follow-up. Conclusion Adrenal angiomyolipomas are rare benign tumours that have the ability to reach a large size and potential to bleed. Here, we report the second case of spontaneous haemorrhage in an adrenal angiomyolipoma, which was successfully treated with open adrenalectomy.

scholarly journals Clozapine-induced stuttering in the absence of known risk factors: a case report

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Florence Jaguga
Keyword(s):  
Risk Factors ◽  
Family History ◽  
Side Effect ◽  
Case Reports ◽  
Extrapyramidal Symptoms ◽  
Prior History ◽  
Case Presentation ◽  
Rare Side Effect ◽  
History Of ◽  
Electrophysiological Findings

Abstract Background Stuttering is a rare side effect of clozapine. It has been shown to occur in the presence of one or more factors such as abnormal electrophysiological findings and seizures, extrapyramidal symptoms, brain pathology, and a family history of stuttering. Few case reports have documented the occurrence of clozapine-induced stuttering in the absence of these risk factors. Case presentation A 29-year-old African male on clozapine for treatment-resistant schizophrenia presented with stuttering at a dosage of 400 mg/day that resolved with dose reduction. Electroencephalogram findings were normal, and there was no clinical evidence of seizures. The patient had no prior history or family history of stuttering, had a normal neurological examination, and showed no signs of extrapyramidal symptoms. Conclusion Clinicians ought to be aware of stuttering as a side effect of clozapine, even in the absence of known risk factors. Further research should investigate the pathophysiology of clozapine-induced stuttering.

scholarly journals Sirenomelia: two case reports

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Asiyeh Shojaee ◽  
Firooze Ronnasian ◽  
Mahdiyeh Behnam ◽  
Mansoor Salehi
Keyword(s):  
Case Reports ◽  
Three Dimensional ◽  
First Trimester ◽  
Mendelian Inheritance ◽  
Lower Limbs ◽  
Congenital Deformity ◽  
White Family ◽  
Her Family ◽  
Caudal Regression ◽  
History Of

AbstractBackgroundSirenomelia, also called mermaid syndrome, is a rare lethal multi-system congenital deformity with an incidence of one in 60,000–70,000 pregnancies. Sirenomelia is mainly characterized by the fusion of lower limbs and is widely associated with severe urogenital and gastrointestinal malformations. The presence of a single umbilical artery derived from the vitelline artery is the main anatomical feature distinguishing sirenomelia from caudal regression syndrome. First-trimester diagnosis of this disorder and induced abortion may be the safest medical option. In this report, two cases of sirenomelia that occurred in an white family will be discussed.Case presentationWe report two white cases of sirenomelia occurring in a 31-year-old multigravid pregnant woman. In the first pregnancy (18 weeks of gestation) abortion was performed, but in the third pregnancy (32 weeks) the stillborn baby was delivered by spontaneous vaginal birth. In the second and fourth pregnancies, however, she gave birth to normal babies. Three-dimensional ultrasound imaging showed fusion of the lower limbs. Neither she nor any member of her family had a history of diabetes. In terms of other risk factors, she had no history of exposure to teratogenic agents during her pregnancy. Also, her marriage was non-consanguineous.ConclusionThis report suggests the existence of a genetic background in this mother with a Mendelian inheritance pattern of 50% second-generation incidence in her offspring.

A PAX-8-positive Female Urethra Adenocarcinoma: A Case Report with Diagnostic Challenges in Intestinal- Type Adenocarcinoma

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S60-S61
Author(s):  
M Torrez ◽  
R Allen ◽  
J Zhou
Keyword(s):  
Pulmonary Nodules ◽  
Urethral Diverticulum ◽  
Vaginal Wall ◽  
Intestinal Type ◽  
Primary Adenocarcinoma ◽  
Female Urethra ◽  
Current Case ◽  
Squamous Mucosa ◽  
Uterine Tumors ◽  
Er Positive

Abstract Introduction/Objective Female urethra adenocarcinoma (FUA) in women is extremely rare, representing 0.02% of all women’s cancers and <1% of cancers in the female genitourinary tract. Intestinal-type primary adenocarcinoma of the urethra is even rarer, with only one documented case to our knowledge. Furthermore, PAX-8 immunoexpression in this entity has not been reported. Here we report an intestinal-type primary urethral adenocarcinoma that developed from inflammation-related metaplasia in urethral diverticulum with positive PAX-8 staining. Methods Clinical chart review and microscopic examination on the lung, urethral, and vaginal wall biopsies and immunohistochemistry were performed. Results A 64-year-old female with a 32-pack-year history of tobacco use was found to have multiple pulmonary nodules on imaging. The tumor cells were positive for CK7, CK20, SAT-B2, and PAX-8 and negative for TTF- 1/Napsin and ER. Positive PAX-8 immunoexpression raised the possibility of a gynecologic/Mullerian primary. Subsequent colonoscopy and imaging showed no evidence of colorectal or uterine tumors. The patient began having hematuria with intermittent urinary retention, and cystoscopy showed a 4 x 3 cm mass involving bladder neck circumferentially and invading into the vaginal wall. Urethral and vaginal tumor biopsies were performed. Morphologic examination of the urethral biopsy demonstrated intestinal metaplasia of squamous mucosa with transition from a mature to dysplastic phenotype where the adenocarcinoma originated from. The vaginal wall biopsy showed the same morphology. The urethral and vaginal wall biopsies showed a similar immunophenotype as the pulmonary nodule biopsy. Conclusion FUA is a rare, aggressive tumor that occurs in Skene’s glands. In our current case, however, it appeared to arise from inflammation-related metaplasia in urethral diverticulum. Another important finding of the case is the positive PAX-8 expression. Therefore in PAX-8 positive tumors, primary adenocarcinoma of lower urinary tract should be in the differential, along with gynecologic/Mullerian tumors.

scholarly journals Severe COVID-19 with acute respiratory distress syndrome (ARDS) in a sickle cell disease adult patient: case report

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Marion Teulier ◽  
Alexandre Elabbadi ◽  
Grigorios Gerotziafas ◽  
François Lionnet ◽  
Guillaume Voiriot ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Case Reports ◽  
Cor Pulmonale ◽  
Point Of View ◽  
Nasopharyngeal Swab ◽  
Prevention Policy ◽  
Cord Injury ◽  
History Of ◽  
Pulmonary Arterial ◽  
Venovenous Ecmo

Abstract Background Sickle-cell anaemia is a widespread genetic disease prevalent worldwide among African and African-American populations. The pathogenesis is most often revealed by pulmonary conditions, including acute thoracic syndrome, which is affecting the life expectancy of these populations. The global spread of CoV2-SARS infection with a respiratory tropism, endothelial damages and procoagulant status endangers the SCD population. However, with only a few case reports, consequences of the Covid-19 pandemic on SCD population remain poorly known. Case presentation We report a case of a 33-year-old man with a history of homozygous SS homozygous sickle cell anemia who consulted on March 24, 2020 for febrile dyspnea 11 days after the onset of symptoms. A nasopharyngeal swab was positive for SARS-CoV-2. His respiratory status worsened rapidly in the emergency room and then in ICU leading to severe ARDS requiring intubation, curarization, and venovenous ECMO. Hematologically, severe hemolysis associated with major thrombocytopenia without documented spinal cord injury was noted. Several transfusion exchanges are performed. The evolution was finally slowly favorable and led to discharge from the intensive care unit and then from the hospital. Conclusions This case recalls the importance of an increased prevention policy against COVID-19among the SCD population. In addition, from a therapeutic point of view, it advocates (1) a high preventive anticoagulation from the outset according to the level of D-dimers (2) the use of venovenous ECMO in this particular case, whereas this technique has had rather disappointing results in acute chest syndromes. (3) Unexpectedly, our patient did not develop pulmonary arterial hypertension (PAH) and acute cor pulmonale (ACP), whereas this is a common feature of ARDS during SCD. These last two observations suggest a different pathophysiology of pulmonary disorders in SCD patients in the case of SARS COv2. It could be associated with marked hypoxemia secondary to pulmonary vascular vasodilation.

scholarly journals Small Cell Carcinoma of the Uterine Cervix in a Pregnant Patient Diagnosed with Liquid Based Cytology and Cell Block Immunocytochemistry

2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Mawuli F. Attipoe ◽  
Charles D. Sturgis
Keyword(s):  
Cell Carcinoma ◽  
Small Cell Carcinoma ◽  
Uterine Cervix ◽  
Case Reports ◽  
Pregnant Patient ◽  
Small Cell ◽  
Tumor Type ◽  
Cell Block ◽  
Current Case ◽  
Small Series

Definitive cytomorphologic diagnosis of small cell carcinoma of the uterine cervix is possible but can be challenging in routine cervicovaginal cancer screening specimens. Several small series of reported cases of cervical small cell carcinoma have shown this uncommon malignancy to represent fewer than 2% of all invasive cervical cancers. This tumor type is associated with poor prognosis and rapid disease progression and can develop to an advanced stage in the interval between screening visits. Only rare case reports of small cell carcinoma arising in gravid cervices are known. In the current case a 29-year-old, gravida 6, para 2, pregnant (10-week gestation) female presented with postcoital bleeding. A definitive diagnosis of small cell carcinoma of the cervix was made possible by liquid based Pap testing with ancillary cell block preparation allowing for immunocytochemical characterization of the lesional cell population.

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