Duplication of the oral cavity and mandible: a rare congenital craniofacial anomaly

A newborn girl was referred to the otolaryngology service after prenatal imaging showed a right mandibular mass. Physical examination revealed a 1–2 cm mass along the right mandible with the appearance of a vestigial oral cavity. Tissue resembling the vermillion and primitive tongue appeared innervated and moved in conjunction with oral movements. MRI and CT of the mandible after birth confirmed a partially ossified soft tissue mass of the right mandibular body, containing unerupted teeth. She was taken to the operating room at 6 months of age for mass excision and reconstruction. Postoperatively, she healed well and was feeding without difficulty. Craniofacial duplication, including duplication of stomatodeal structures or diprosopus, is a rare condition with a variety of phenotypes. In the case of suspected craniofacial duplication, associated syndromes should be ruled out and appropriate imaging employed to determine the extent of involvement of adjacent structures, which will ultimately guide surgical planning.

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Agenesis of the Right Hepatic Lobe

Case Reports in Medicine ◽

10.1155/2012/415742 ◽

2012 ◽

Vol 2012 ◽

pp. 1-3 ◽

Cited By ~ 3

Author(s):

Lucas Souto Nacif ◽

Yuri dos Santos Buscariolli ◽

Luiz Augusto Carneiro D'Albuquerque ◽

Wellington Andraus

Keyword(s):

Surgical Planning ◽

Rare Condition ◽

Middle Hepatic Vein ◽

Developmental Defects ◽

Hepatic Lobe ◽

Right Lobe ◽

Loss Of Appetite ◽

The Right ◽

Previous Disease ◽

Rare Diagnosis

Introduction. Agenesis of the right lobe of the liver is a rare finding and was defined as the absence of liver tissue on the right side, with preservation of the middle hepatic vein, without previous disease or surgery. It is usually an incident finding reveled by imaging exams or during abdominal surgery.Case Report. A 32-year-old male patient was admitted to the hospital for abdominal discomfort and loss of appetite. Imaging studies revealed the absence of the right hepatic lobe and hypertrophied left hepatic segments.Discussion. Anomalies of hepatic morphology are rare and correspond to developmental defects during embryogenesis, are a rare diagnosis, and are generally diagnosed incidentally based on imaging. Agenesis or hypoplasia of the right lobe may predispose the patient to the development of portal hypertension and esophageal varices. Surgical knowledge of such anatomical agenesis is necessary for surgical planning, for the appropriate identification of intraoperative surgical findings, and for the design of the postoperative approach to therapy.Conclusion. Agenesis of the right hepatic lobe is a rare condition. We want to highlight the importance of understanding the condition. Surgeons must recognize the entity in order to deal appropriately with the findings.

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Pleomorphic giant cell adeno-carcinoma gall bladder: a histological rarity and a surgical dilemma

International Surgery Journal ◽

10.18203/2349-2902.isj20171171 ◽

2017 ◽

Vol 4 (4) ◽

pp. 1497

Author(s):

M. S. Ray ◽

B. S. Deepak ◽

Aneeta Jassar ◽

Kunjan Pathania

Keyword(s):

Gall Bladder ◽

Giant Cell ◽

Rare Condition ◽

Liver Parenchyma ◽

Undifferentiated Carcinoma ◽

Treatment Modalities ◽

Tissue Mass ◽

Upper Abdominal ◽

The Right ◽

Adeno Carcinoma

Undifferentiated pleomorphic giant cell adeno-carcinoma of the gall bladder (GB) is an extremely rare condition and is usually an incidental diagnosis. Here, we describe a rare case of gall bladder carcinoma in a 50 year old lady who presented with upper abdominal pain and palpable lump in the right hypochondrium. Computed tomography and Ultrasonography of the abdomen showed an ill-defined giant heterogeneous soft tissue mass involving gall bladder and adjacent liver parenchyma with obvious regional adenopathy. Metastatic work-up was abnormal and the patient underwent radical cholecystectomy. Histopathology and immunohistochemistry (IHC) study revealed Undifferentiated carcinoma or pleomorphic giant cell adenocarcinoma of gall bladder involving the adjacent hepatic parenchyma. Patient received post-op adjuvant chemotherapy and is under monthly follow-up. The present case emphasizes the need for better detection, evaluation and analysis of such rare entities, to identify their natural course and effective treatment modalities.

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An atypical presentation of Ormond’s disease

Portuguese Journal of Nephrology & Hypertension ◽

10.32932/pjnh.2021.12.160 ◽

2022 ◽

Vol 35 (4) ◽

Author(s):

João Carvão ◽

◽

Carlota Vida ◽

Luís Resende ◽

Francisca Silva ◽

...

Keyword(s):

Retroperitoneal Fibrosis ◽

Immunohistochemical Study ◽

Rare Condition ◽

Therapeutic Strategies ◽

Atypical Presentation ◽

Ormond’S Disease ◽

Randomized Controlled ◽

Adjacent Structures ◽

The Right ◽

Inflammatory Tissue

Retroperitoneal fibrosis is a rare condition defined by an overproduction of fibro-inflammatory tissue in the retroperitoneum, usually involving the abdominal aorta, but also managing to invade adjacent structures. We report a case of a 47-year-old female patient with an atypical radiological presentation of retroperitoneal fibrosis, involving predominantly the right kidney. Ultimately, the diagnosis was performed by an immunohistochemical study that identified IgG4+ plasmocytes after kidney nephrectomy, also known as Ormond’s disease. There are no universal guidelines for the treatment of retroperitoneal fibrosis, due to its rarity and lack of randomized controlled trials comparing different therapeutic strategies.

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CLINICAL CASE OF A MALIGNANT PECOMA OF THE LUNG

Problems in oncology ◽

10.37469/0507-3758-2019-65-5-756-759 ◽

2019 ◽

Vol 65 (5) ◽

pp. 756-759

Author(s):

Mikhail Postolov ◽

Nadezhda Kovalenko ◽

K. Babina ◽

Stanislav Panin ◽

Yelena Levchenko ◽

...

Keyword(s):

Good Condition ◽

Lung Neoplasm ◽

Rare Condition ◽

Epithelioid Cell ◽

X Ray ◽

Perivascular Epithelioid Cell ◽

Mesenchymal Neoplasm ◽

Preoperative Ct ◽

Lymph Duct ◽

The Right

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm characterized by expression of both mela-nocytic and smooth muscle markers. Only 10 primary malignant lung PEComas have been reported up to date. We report a 59-year-old male who presented with a lung neoplasm, found during the routine X-ray examination. Preoperative CT-scan revealed the 3,5-cm-sized mass, located at the border of the upper, middle and lower lobes of the right lung. Patient underwent a thoracotomy, resection of the upper, middle and lower lobes of the right lung accompanied with mediastinal lymphadenectomy. After surgery, chylothorax was revealed. Conservative treatment was unsuccessful, so we performed laparoscopic clipping of the thoracic lymph duct. Patient was dismissed from hospital on the 10-th day after the second operation in good condition. In this report, we intend to increase the limited knowledge relating to natural history and optimal treatment of such a rare condition as a primary malignant lung PEComa.

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Fibroadenoma of Ectopic Breast Tissue in the Groin – A Rare Disease Entity

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqaa161.101 ◽

2020 ◽

Vol 154 (Supplement_1) ◽

pp. S47-S48

Author(s):

D Emechebe ◽

M Alshal ◽

T Rana ◽

M Agaronov

Keyword(s):

Breast Tissue ◽

Tissue Mass ◽

Ectopic Breast Tissue ◽

The Common ◽

Painless Mass ◽

The Right ◽

Ectopic Breast ◽

Urogenital Anomalies ◽

Superficial Soft Tissue

Abstract Introduction/Objective Ectopic breast tissue (EBT) is a well-documented anomaly of the breast and commonly presents along the embryonic milk line extending between the axilla and groin. Reported incidence of accessory breast is 0.4–6% in females. Pathologies developing in an EBT are reported as a rare entity in the literature. Carcinoma is reported as the common pathology followed by inflammation and fibroadenoma Methods We present a case of 43-year- old female who presented with a painless mass in her right groin for the past year which gradually increased in size. CT abdomen pelvis with contrast showed a 2.2 x 3.0 x 4.4 cm superficial soft tissue mass in the right groin which was suspected to be a lymph node. Further investigation and histopathological report of biopsy showed ectopic breast tissue with admixed chronic inflammation and reactive changes.However, excision of the mass three months later showed showed proloferation of both glandular and stromal elements. Results The tissue from the biopsy was positive for GATA 3, mammoglobin, GCDFP and CD 10 and the histological features on excision was confirmatory of fibroadenoma. Conclusion In conclusion, when tumors or nodules are found along the mammary line, the presence of breast tissue should be considered during the investigation. It is clinically wise to evaluate and screen carefully cases of supernumerary breast for any pathology and for any associated urogenital anomalies such as supernumerary kidneys, polycystic kidneys and renal cell adenocaricnoma. In our case, patient had no associated urogenital anomalies and she is on follow up.

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Rheology of right ventricular outflow tract obstruction: sub-pulmonary membrane developing months after primary intervention to treat pulmonary atresia with intact interventricular septum

Cardiology in the Young ◽

10.1017/s1047951121001591 ◽

2021 ◽

pp. 1-4

Author(s):

Baher M. Hanna ◽

Wesam E. El-Mozy ◽

Sonia A. El-Saiedi

Keyword(s):

Right Ventricular ◽

Interventricular Septum ◽

Pulmonary Atresia ◽

Right Ventricular Outflow Tract ◽

Rare Condition ◽

Surgical Excision ◽

Ventricular Outflow Tract ◽

Outflow Tract ◽

Ventricular Outflow Tract Obstruction ◽

The Right

Abstract Isolated sub-pulmonary membrane is a rare condition, the origin of which has been debatable. Transcatheter treatment of pulmonary valve atresia with intact interventricular septum by radiofrequency perforation and balloon dilatation to restore biventricular circulation is gaining more popularity, with improving results over time. We report in our experience of 79 cases in 10 years the development of a sub-pulmonary membrane in 4 cases: causing significant obstruction requiring surgical excision in one case that revealed a fibrous membrane on pathology; causing mild right ventricular outflow tract obstruction in another and not yet causing obstruction in 2. On cardiac MRI, the right ventricular outflow tract and the right ventricular outflow tract/pulmonary atresia angle showed no morphological abnormalities.

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Acute otitis media in an infant complicated with osteoarthritis of temporomandibular joint and sub-periosteal abscess of the clavicle

The Egyptian Journal of Otolaryngology ◽

10.1186/s43163-021-00123-8 ◽

2021 ◽

Vol 37 (1) ◽

Author(s):

Kaoutar Cherrabi ◽

Hind Cherrabi

Keyword(s):

Septic Arthritis ◽

Temporomandibular Joint ◽

Nutritional Assessment ◽

Acute Otitis Media ◽

Clinical Signs ◽

Rare Condition ◽

Pediatric Emergency ◽

Ear Infections ◽

The Right

Abstract Background Otomastoiditis is a very frequent affection and a current complication of mal-treated benign ear infections in children. However, this a very rare case of the association of two rare complications of otomastoiditis in a newborn. On the one hand, septic arthritis of the temporomandibular joint which is a very rare condition that is difficult to diagnose, and when unrecognized or not treated accordingly, it can resolve in serious infectious complication and or definitive injury to the temporomandibular joint. On the other hand, osteomyelitis of the clavicle is also very rare, and only a few cases have been cited in the literature concerning infants. Case presentation This 46-day-old infant was brought to pediatric emergency consultation for 2 swelling inflammatory bulges, one in the right mastoid and pre-auricular regions, and another in the right basi-cervical area. The infant was hypertrophic febrile, hypotonic, and pale. He had preserved archaic reflexes. Besides, blood test showed an inflammatory syndrome, inflammatory anemia, and no other abnormalities. Upon supplementary computed tomodensitometry exam, the diagnosis of a combination of septic arthritis of the right temporomandibular joint and sub-periosteal abscess of the ipsilateral clavicle in a context of hypotrophy and malnutrition was suspected. A pus sample was obtained for bacteriological evaluation, after which the infant had a course of intravenous associated antibiotics, along with nutritional assessment and management. Surgical drainage of both collections was performed. The 6-month follow-up was satisfactory, without clinical signs of functional impact on temporomandibular joint, or acromioclavicular joint. Conclusion This work stresses the necessity of thorough clinical examination of infants even in cases of benign ear infections, as well as the importance of adapted treatment and follow-up, which could allow early diagnosis, appropriate treatment, or even prevention of severe complications that can be associated with such benign conditions.

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Hyperthermia in a pediatric patient with neuroblastoma during anesthesia: a case report

BMC Surgery ◽

10.1186/s12893-021-01124-3 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Chuan Wang ◽

Wenqiong Xin ◽

Yi Ji

Keyword(s):

Tumor Resection ◽

Neuron Specific Enolase ◽

Tracheal Extubation ◽

Posterior Mediastinum ◽

Gas Analysis ◽

Vanillylmandelic Acid ◽

Management Options ◽

Paravertebral Space ◽

Tissue Mass ◽

The Right

Abstract Background Neuroblastoma is the most common malignant extracranial solid tumor in pediatrics patients. Intraoperative hyperthermia is extremely rare in patients with neuroblastoma and can cause a series of complications. Here, we represent a case of neuroblastoma accompanied by hyperthermia during anesthesia, and propose a rational explanation and management options. Case presentation The patient had gait disturbance and sitting-related pain without fever. Magnetic resonance imaging revealed a soft tissue mass located in the right posterior mediastinum, paravertebral space and canalis vertebralis. Serum tumor marker screening showed that the patient had increased epinephrine, norepinephrine and neuron specific enolase levels, with an increased 24 hour urine vanillylmandelic acid level. Intraspinal tumor resection was conducted. The temperature of the patient rapidly arose to 40.1 °C over 10 minutes when waiting for tracheal extubation. The arterial gas analysis results indicated malignant hyperthermia was less likely, and dantrolene was not administered. Physical cooling methods were used, and the temperature dropped to 38.6 ℃. The trachea was successfully extubated. Histological results confirmed the diagnosis of neuroblastoma. Conclusions Hyperthermia during anesthesia is a serious adverse event. Catecholamines secreted from neuroblatoma cells can lead to hypermetabolism and hyperthermia. Surgeons and anesthesiologists should be aware of the possibility of hyperthermia in patients with neuroblastoma.

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P043 Fascinating phosphate: seek and you will find

Rheumatology ◽

10.1093/rheumatology/keab247.040 ◽

2021 ◽

Vol 60 (Supplement_1) ◽

Author(s):

Jyoti Bakshi ◽

Clare Batten

Keyword(s):

Back Pain ◽

Degenerative Change ◽

Nasal Bone ◽

Rare Condition ◽

Whole Body ◽

Excision Biopsy ◽

Small Lesion ◽

History Of ◽

The Right ◽

Fgf 23

Abstract Background/Aims A 62-year-old accountant was referred to the metabolic bone clinic with a 2 year history of thoracic back pain and a persistently raised ALP. There were no associated red flags for her back pain. She had a history of a gluteal lump, thought to be benign, for which she had declined excision. She has hypertension and had a previous navicular fracture. Medications included bendroflumethiazide and Adcal D3. She had restriction in neck movements and was tender to percussion in the thoracic spine. There was no proximal weakness or focal neurology. Systems and joint exam were unremarkable. Methods The case is discussed below. Results Salient abnormal results on presentation were a raised ALP of 207 and corrected calcium of 2.34. PTH was elevated at 8.2 (NR:1.6-6.9), Vitamin D 79 and a low phosphate of 0.34 (NR:0.8-1.50). Alkaline phosphatase isoenzymes showed the raised level came from bone. Protein and urine electrophoresis were normal. A bone density scan was normal, and a recent thoracic MRI showed only degenerative change. An isotope bone scan was requested and was reported to show increased activity in the nasal bone, maxilla and both orbits, raising the possibility of Paget’s disease. However, when reviewed in the Radiology meeting with a skull x-ray, the appearances were not felt to be in keeping with Paget’s. Despite physiotherapy, hydrotherapy, acupuncture and neuropathic medication the patient’s back pain continued. Her phosphate remained low and her calculated tubular reabsorption of phosphate from a 24h urine collection (TmP/GFR) was low at 0.42mmol/l (NR 0.80-1.35). She was started on phosphate replacement and calcitriol, and Adcal D3 was continued. The Fibroblast Growth Factor (FGF) 23 levels were sent and came back significantly elevated at 1380 (NR < 100). A 68Ga DOTA-TATE scan (whole body PET/CT scan), confirmed the right gluteal lump as the source of the FGF 23. The patient went on to have an excision biopsy and histology confirmed a mesenchymal tumour of the right buttock. Her phosphate replacement was gradually weaned, but on reducing the dose phosphate levels dropped and her symptoms returned. The repeat TmP/GFR was again low at 0.61, and FGF 23 levels were still raised at 204. A repeat 68Ga DOTA-TATE scan, 4 years after the first one, showed recurrence of the right gluteal lesion and a possible small lesion in the left gluteal muscle. She has been sent for further excision. Conclusion Tumour induced osteomalacia (TIO) is a rare condition and should be considered in cases of hypophosphataemia. Classical symptoms are proximal weakness and muscle and bone pain. They are typically associated with small benign tumours (most commonly mesenchymal tumours) which may be difficult to find. Excision is curative but if small amounts of tumour remain, relapses may occur. Disclosure J. Bakshi: None. C. Batten: None.

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Chalastospora gossypii in a Maine Coon cat: case report and literature review

Journal of Veterinary Diagnostic Investigation ◽

10.1177/10406387211022298 ◽

2021 ◽

pp. 104063872110222

Author(s):

Samantha M. Norris ◽

Paula A. Schaffer ◽

Noah B. Bander

Keyword(s):

Soft Tissue ◽

Needle Aspiration ◽

Tissue Mass ◽

Large Numbers ◽

Formalin Fixed Paraffin ◽

Formalin Fixed Paraffin Embedded ◽

Soft Tissue Masses ◽

Subcutaneous Mass ◽

The Right ◽

A Minor

A 15-y-old castrated male Maine Coon cat was evaluated for an ulcerated soft tissue mass on the right hindlimb that had been observed for 4 mo and had grown rapidly. A 3 × 3 cm soft, raised, amorphous, and ulcerated subcutaneous mass was observed on the lateral right metatarsus. In-house cytology via fine-needle aspiration was nondiagnostic. Incisional biopsy of the mass and further staging was declined, and amputation was elected. The amputated limb was submitted for histopathology, which revealed severe chronic nodular granulomatous dermatitis and multifocal granulomatous popliteal lymphadenitis with large numbers of intralesional fungal hyphae. Fungal PCR and sequencing on formalin-fixed, paraffin-embedded tissue identified Chalastospora gossypii. No adjunctive therapy was elected at the time. The patient has done well clinically 1 y post-operatively. C. gossypii is a rare microfungus found worldwide and is considered a minor pathogen of several plants. To our knowledge, infection by this fungus has not been reported previously in veterinary species. Features in our case are comparable to other mycotic infections. Nodular granulomatous mycotic dermatitis and cellulitis, although uncommon, should be a differential for soft tissue masses in veterinary species; C. gossypii is a novel isolate.

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