scholarly journals Systemic congenital lymphangiomatosis

1996 ◽  
Vol 114 (5) ◽  
pp. 1278-1281 ◽  
Author(s):  
Ligia Maria Suppo de Souza ◽  
Maria Regina Bentlin ◽  
Eliana Souto de Abreu ◽  
Carlos Eduardo Bacchi
Keyword(s):  
Young Adults ◽  
Respiratory Failure ◽  
Rare Disease ◽  
Poor Prognosis ◽  
Rare Case ◽  
Soft Tissues ◽  
Lymphatic Channel ◽  
Autopsy Findings ◽  
Children And Young Adults ◽  
Cervical Area

Systemic lymphangiomatosis is a rare disease characterized by the exageration of lymphatic channel proliferation, occurring in children and young adults. We describe an extremely rare case of congenital systemic lymphangiomatosis in a newborn who had ascitis and respiratory failure develop immediately after delivery. Death occurred during the first hour of life. Autopsy findings showed numerous cysts in soft tissues of the cervical area, mediastinum and diaphragm, and several other organs including the liver, spleen, thyroid and kidneys. The severe and diffuse involvement with cysts in both lungs by lymphangiomatosis was associated with poor prognosis and death in our case.

CLEAR CELL SARCOMA OF SOFT TISSUES IN CHILDREN AND YOUNG ADULTS: The St. Jude Children's Research Hospital Experience

1999 ◽  
Vol 16 (6) ◽  
pp. 539-544 ◽  
Author(s):  
Sudha Parasuraman ◽  
Bhaskar N. Rao ◽  
Sara Bodner ◽  
Alvida Cain ◽  
Charles B. Pratt ◽  
...  
Keyword(s):  
Young Adults ◽  
Soft Tissues ◽  
Clear Cell ◽  
Clear Cell Sarcoma ◽  
Cell Sarcoma ◽  
Research Hospital ◽  
Hospital Experience ◽  
Children And Young Adults

scholarly journals Dysembryoplastic neuropithelial tumor: a rare case report

2017 ◽  
Vol 5 (5) ◽  
pp. 2270
Author(s):  
Shailesh Thanvi ◽  
Hemant Jangid ◽  
Yogi Raj Joshi
Keyword(s):  
Young Adults ◽  
Case Report ◽  
Rare Case ◽  
Male Child ◽  
Adjuvant Chemoradiotherapy ◽  
Rare Entity ◽  
Intractable Seizures ◽  
Rare Case Report ◽  
Children And Young Adults ◽  
Space Occupying Lesion

Dysembryoplastic neuropithelial tumor (DNET) is a rare recently described, benign glioneural tumor frequently associated with intractable seizures in children and young adults which is important to recognise clinically and radiologically as it is surgically curable without need for adjuvant chemoradiotherapy. We hereby present a case report of a 10year old male child who presented with intractable seizures and right parietal space occupying lesion which was diagnosed DNET radiologically, treated by microsurgical excision and confirmed histopathologically as DNET, thus emphasising multidisciplinary role in management of this rare entity.

scholarly journals Primitive neuroectodermal tumor of ulnar nerve: A rare case

2021 ◽  
Author(s):  
Mohammadreza Emamhadi ◽  
Sama Noroozi Guilandehi ◽  
Nooshin Zaresharifi ◽  
Iraj Baghi ◽  
Alireza Mehrvarz ◽  
...  
Keyword(s):  
Young Adults ◽  
Ulnar Nerve ◽  
Peripheral Nerves ◽  
Rare Case ◽  
Primitive Neuroectodermal Tumor ◽  
Neuroectodermal Tumor ◽  
Children And Young Adults ◽  
Aggressive Tumor

Primitive neuroectodermal tumor (PNET) is a highly aggressive tumor and mostly develops in children and young adults. PNETs of peripheral nerves are uncommon. Ulnar nerve, in particular, is an extremely peculiar origin for PNET and to the best of our knowledge only few well-documented cases have been yet reported.

scholarly journals Mitotically Active Plexiform Fibrohistiocytic Tumor

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Ebru Zemheri ◽  
Şeyma Özkanlı ◽  
Serkan Şenol ◽  
Filiz Ozen ◽  
Cigdem Ulukaya Durakbaşa ◽  
...  
Keyword(s):  
Young Adults ◽  
Local Recurrence ◽  
Soft Tissues ◽  
Upper Extremities ◽  
Mesenchymal Neoplasm ◽  
Systemic Metastasis ◽  
Children And Young Adults ◽  
Plexiform Fibrohistiocytic Tumor ◽  
Slow Growing

Plexiform fibrohistiocytic tumor is an intermediate malignant tumor situated in superficial soft tissues. It affects children and young adults. The tumor is most commonly located on upper extremities, whereas involvement of back region is rare. Mitotic activity is generally low (~3/10 HPF). It is rare, but it can exhibit aggressive behavior, so total excision with clear surgical margins and long-term followup is necessary to detect local recurrence and metastases. We report a child with a solid mass on back region which was found to be a mitotically active plexiform fibrohistiocytic tumor (6/10 HPF) after excision. Plexiform fibrohistiocytic tumor (PFT) is a mesenchymal neoplasm of children, adolescents, and young adults. It is characterized by fibrohistiocytic cytomorphology and multinodular growth pattern. Clinically it is usually a slow-growing mass of upper extremities with frequent local recurrence and rare regional lymphatic and systemic metastasis (Fletcher et al. (2002), Enzinger and Zhang (1988), Remstein et al. (1999)).

scholarly journals Antrochoanal polyp with bilateral extension, bony erosion and protruding out through anterior nares in 45 year old female: rare case presentation

2016 ◽  
Vol 2 (2) ◽  
pp. 99
Author(s):  
Sonia Jindal ◽  
Pooja Arya ◽  
Deepchand Lal ◽  
Ramesh Kadela ◽  
Gourav Gupta ◽  
...  
Keyword(s):  
Young Adults ◽  
Nasal Cavity ◽  
Rare Case ◽  
Medial Wall ◽  
Antrochoanal Polyp ◽  
Left Nostril ◽  
Solitary Lesion ◽  
Case Presentation ◽  
Children And Young Adults ◽  
Bony Erosion

<p class="abstract">Antrochoanal polyp or Killian polyp is a benign, solitary lesion that affects mainly children and young adults. It is usually unilateral in occurrence without bony erosion. We report a unilateral antrochoanal polyp of left side, in 45 year old female, extending into nasopharynx, right sided nasal cavity, protruding out through left nostril and causing bony erosion of medial wall of maxilla on left side.</p>

scholarly journals Effective Initial Treatment of Diffuse Pulmonary Lymphangiomatosis with Sirolimus and Propranolol: A Case Report

Medicina ◽  
2021 ◽  
Vol 57 (12) ◽  
pp. 1308
Author(s):  
Ieva Dimiene ◽  
Kristina Bieksiene ◽  
Jurgita Zaveckiene ◽  
Mindaugas Andrulis ◽  
Daiva-Elzbieta Optazaite ◽  
...  
Keyword(s):  
Young Adults ◽  
Case Report ◽  
Rare Disease ◽  
Initial Treatment ◽  
Benign Disease ◽  
Diagnosis And Treatment ◽  
Clinical Effects ◽  
Children And Young Adults

Diffuse pulmonary lymphangiomatosis (DPL), an exceptionally rare disease, mainly occurs in children and young adults of both sexes. Even though DPL is considered to be a benign disease, its prognosis is relatively poor. Because of its rarity, little guidance on diagnosis and treatment is available, which makes working with patients with DPL challenging for clinicians. We present here a case of a young man with DPL in whom treatment with sirolimus and propranolol rapidly achieved positive radiological and clinical effects.

scholarly journals Peripheral primitive neuroectodermal tumor of the small bowel mesentery: Report of a case

2015 ◽  
Vol 143 (9-10) ◽  
pp. 619-622
Author(s):  
Helena Maric ◽  
Radovan Cvijanovic ◽  
Igor Ivanov ◽  
Ljiljana Gvozdenovic ◽  
Dejan Ivanov ◽  
...  
Keyword(s):  
Young Adults ◽  
Small Intestine ◽  
Primitive Neuroectodermal Tumor ◽  
Soft Tissues ◽  
Clinical Presentation ◽  
Neuroectodermal Tumor ◽  
Small Bowel Mesentery ◽  
Peripheral Primitive Neuroectodermal Tumor ◽  
Neural Origin ◽  
Children And Young Adults

Introduction. Primitive neuroectodermal tumor or Ewing?s sarcoma is a tumor of undifferentiated small round cells that arise from the soft tissues, and is believed to be of neural origin. It occurs most often in children, followed by adolescents and young adults. Case Outline. A case of a 24-year-old patient with ulcerostenosans Ewing?s sarcoma of the initial part of the small intestine is presented in our paper. Reviewing the literature and using as an example the case of a female patient with signs of sideropenic anemia caused by primitive neuroectodermal tumor of the small intestine, an attempt was made to clarify the etiology, clinical presentation, diagnosis and therapy with the aim of its rapid detection and treatment. Conclusion. Mesenteric primitive neuroectodermal tumor is a rare neoplasm in adults, while it usually occurs in children and young adults. Surgical resection of the lesions with the application of chemotherapy is the main form of treatment of patients suffering from this disease.

scholarly journals Malignant tumors of the kidney, brain, and soft tissues in children and young adults with the tuberous sclerosis complex

Cancer ◽  
1998 ◽  
Vol 83 (10) ◽  
pp. 2208-2216 ◽  
Author(s):  
Tahseen Al-Saleem ◽  
Lisa L. Wessner ◽  
Bernd W. Scheithauer ◽  
Kathleen Patterson ◽  
E. Steven Roach ◽  
...  
Keyword(s):  
Young Adults ◽  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Soft Tissues ◽  
Malignant Tumors ◽  
Children And Young Adults
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