Multiple Cutaneous Granular Cell Tumors: Case Report of a 19-Year-Old African American Female

2011 ◽  
Vol 15 (6) ◽  
pp. 344-346 ◽  
Author(s):  
Matthew Henry ◽  
Adam Perry

Background and Objective: This is a somewhat rare case of a 19-year-old African American female with multiple cutaneous granular cell tumors. Granular cell tumors are of neural origin, except in rare cases, and are considered benign, with a low incidence of malignancy. The clinical presentation varies greatly, but these tumors are most commonly painful and slow growing, with two-thirds occurring on the head and neck. Patients are most commonly in their second to fourth decades of life, two-thirds are black, and two-thirds are women. Granular cell tumors are diagnosed by the characteristic pathologic findings of polygonal cells with eosinophilic granular cytoplasm. Conclusion: These tumors are most commonly singular but can be multiple in 10 to 15% of patients. Older patient age, rapid growth or enlargement, and a history of local recurrence should raise concern for malignant behavior. The distribution and family history in this case are suggestive of possible mosaicism.

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Fabiola D’Ambrosio ◽  
Isabella Marranzini-Rodriguez ◽  
Roxana Aguirre Castaneda ◽  
Claudia Boucher-Berry

Abstract INTRODUCTION: We present 2 pediatric patients with Hyperosmolar Hyperglycemic State (HHS) at diabetes onset. CASE 1: 3 year old African American female presented to the pediatrician office with a 5 day history of polydipsia, polyuria and emesis. POCT glucose read high and patient was transferred to the PICU. Laboratory studies were significant for serum glucose of 1032 mg/dl, Na 128 mMOL/L (corrected 142mMOL/L), VBG showed pH 7.36, HCO3 20 mMOL/L, Serum osm 331 mOsm/Kg. Patient received a 20ml/kg bolus of 0.9%NaCl, followed by 2 times maintenance IV fluids and glargine 2 units. Glucose dropped from 418 to 122 mg/dl in 3 hours. Due to this Dextrose was added and IVF rate was decreased. CASE 2: 8 year old African American obese male was admitted to the PICU for management of new onset diabetes. He presented with 3 days of flu-like symptoms and worsening drowsiness. Patient had increased consumption of large quantities of sugary beverages due to increased thirst. Laboratory workup: serum glucose of 2309 mg/dl, Na 133 mMOL/L (corrected 168 mMOL/L), pH 7.13, HCO3 10 mMOL/L. Patient was given 30 cc/kg NS bolus followed by an insulin drip of 0.1 u/kg/hour. Repeat studies 3 hours later showed a serum glucose of 1,414 mg/dl, Na 152 mMol/L (corrected 184 mMOL/L), pH 7.19, HCO3 17 mMOL/L, and serum osmolality of 408 mOsm/Kg. IVF were adjusted to correct the water deficit and insulin drip was decreased to 0.05u/kg/hour. DISCUSSION: HHS continues to be a challenging diagnosis due to its low frequency compared with Diabetic Ketoacidosis especially when presenting at a very young age. Most practitioners will mistake the presentation for DKA and start an insulin drip. The early use of insulin is not necessary in the setting of HHS due to the risk of complications. A fast drop in glucose decreases the osmotic pressure and compromises the circulatory status with a higher chance of thromboembolism. In mixed HHS and DKA, the management aligns more with the DKA management but the amount of fluids needed is higher and insulin infusion may cause fast drop of glucose with potential decrease of intravascular volume as in our second patient. It is imperative that the diagnosis of HHS is made early so that the appropriate treatment can be instituted. CONCLUSION: Appropriate fluid administration and delay in insulin administration are key in the management of HHS. The awareness of this possible presentation and the early recognition and appropriate fluid management are needed to improve outcomes. REFERENCE: Zeitler, Phil, et al. “Hyperglycemic Hyperosmolar Syndrome in Children: Pathophysiological Considerations and Suggested Guidelines for Treatment.” The Journal of Pediatrics, vol. 158, no. 1, 2011, doi:10.1016/j.jpeds.2010.09.048


2016 ◽  
Vol 4 (1) ◽  
Author(s):  
Alexander H. Cho ◽  
Jessica Logan ◽  
Jalil Ahari

A 24-year-old African American female nonsmoker, with a reported history of asthma presented to the hospital with 2 weeks of shortness of breath and sputum production. She had a chest X-ray and computed tomography scan that displayed evidence of a right upper lobe collapse. She subsequently had a bronchoscopy that revealed an endobronchial lesion at the opening of the right upper lobe bronchus. Biopsies performed were consistent with a granular cell tumor. Granular cell tumors are rare submucosal tumors of Schwann cell origin. Resection of the right upper lobe resolved her symptoms and wheezing.


2021 ◽  
Author(s):  
Bruno Custódio Silva ◽  
Hugo Roberto Sampaio Martins ◽  
Thais Vanessa Salvador ◽  
Guilherme Parmigiani Bobsin ◽  
Paulo Ricardo Gazzola Zen ◽  
...  

Introduction: Chromosome 18 trisomy or Edwards syndrome (ED) is characterized by wide clinical manifestations, usually associated with neurological symptoms and a poor prognosis. Objective, materials and methods: Describe the clinical findings, especially the neurological ones, of a sample of patients with mosaic chromosome 18 trisomy. These were evaluated at a Clinical Genetics Service from 1975 to 2008. Results: During the study, 50 patients with ED were diagnosed, 5 of them (10%) in mosaic. The average number of cells analyzed in these cases was 27,8. Three of the 5 patients (60%) were male. The age at evaluation ranged from 14 to 5926 days (median 93 days). The small number of clinical findings described was noteworthy, both in the dysmorphological evaluation and complementary exams. The main changes were micrognathia (n = 3), low ears implanted (n = 2), retroverted ears (n = 3), clenched fists with overlapping toes (n = 2) and clubfoot (n = 1). As for internal organs, congenital heart disease was reported in 2 cases (40%). All patients had a history of delayed neuropsychomotor development. The older patient also had a description of cognitive impairment and seizures. Conclusions: The clinical presentation of our patients is consistent with what is described in the literature, since they point out due to small number of changes. However, the delay in neuropsychomotor development and neurological symptoms are constant findings. Thus, pediatricians and neurologists should be aware of this possibility.


2022 ◽  
pp. 42-44
Author(s):  
Danielle C. Ware

A 61-year-old African American female presents to an outpatient family health center with a hyperpigmented nodular rash of 2 months’ duration. The rash first appeared on her abdomen before spreading across her upper arms, lower leg, back, face and scalp. She has a history of controlled type 2 diabetes mellitus, cerebral aneurysm rupture, Sjögren’s syndrome, asthma and a left below-the-knee amputation due to osteomyelitis. She smokes cigarettes but does not use alcohol or illicit substances. She has also noticed a dry cough with mild dyspnea on exertion over the past 6 months. On physical exam, hyperpigmented nodules are palpable in both the intradermal and subcutaneous layers of the skin. Nodules are firm, mobile and nontender. Alopecia is noted where scalp nodules are present. Her lungs exhibit diminished air movement throughout, with scattered, end-expiratory wheezing.


Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4011-4011
Author(s):  
Randi J. Katz ◽  
Amir Steinberg ◽  
Robert Klafter

Abstract Factor VII deficiency is a rare autosomal recessive disorder. Its incidence is thought to be 1 in 500,000. Symptoms vary from mild to severe. Factor VII deficient patients usually do not experience bleeding if their level of factor VII is less than 10%. Manifestations are seen with levels less than 5%, and severe bleeding can occur with levels less than 1%. Surgical hemostasis is obtained with levels greater than 25%. Factor VII deficiency is notable for causing a prolonged PT with a normal aPTT, making the diagnosis easy to determine. This is a descriptive study based on a population of patients from our community based teaching hospital with severe Factor VII deficiency. We reviewed lab data and found any patients with less than a 5% factor deficiency from March 2004- June 2006. In our experience we have noted varying manifestations in moderate factor deficiency patients. We describe four patients with varying symptoms, all with factor levels less than 3%. Their charts were reviewed, and three of the patients were available for a telephone interview. Patient A is a 75 yo African American female found to have factor deficiency after an increased Prothrombin time (PT) of 31.2 seconds, INR 5.9, and Partial thromboplastin time (PTT) of 34.9 seconds on routine blood work. This patieny’s factor level was found to be 1.86%. Patient A denied any history of bleeding complications, including an uncomplicated tooth extraction. Patient B is a 46 yo African American female with a long history of bleeding complications. Laboratory data revealed a PT of 26 seconds, INR 4.2, PTT 22 seconds and a Factor level of 1%. She experienced years of heavy menstrual periods and nose bleeds. A hysterectomy was performed secondary to bleeding fibroids. She required multiple doses of novoseven as well as FFP. Patient C was diagnosed at the age of 11. Laboratory results revealed a factor level of 1.87 %, and a PT of 25.1 seconds and an INR of 4.1. She has had minor bleeding events, such as gum bleeding. She did receive FFP prior to a foot surgery, and tooth extraction. Patient D is a 46 year old Hispanic man found to have and INR of 4.3 and, factor VII 1.45. Pt had an uncomplicated cholocystectomy. This is a descriptive study of four different patients with factor VII levels of less than 2%. These four cases demonstrate the wide range of clinical manifestations that factor VII patients may experience. One patient did require novoseven prior to a hysterectomy. Though, as our cases illustrate, some patients do not experience any bleeding manifestations, and therefore, prophylactic plasma may not be required. Furthermore, treatment of factor VII deficient patients needs to be individualized, and guided by personal history. Coagulation Studies of our Patients Patient Prothrombin Time INR Factor VII A 31.2 5.9 1.86% B 26 4.2 1% C 25.1 4.1 1.87% D 46 4.3 1.45%


2001 ◽  
Vol 4 (1) ◽  
pp. 89-93 ◽  
Author(s):  
Andrew H. Chang ◽  
Walter E. Kaufmann ◽  
Daniel J. Brat

A 4-month-old infant with a history of nasopharyngeal teratoma developed progressive optic neuropathy. Neuroimaging studies demonstrated a solid, isointense, suprasellar mass impinging on optic nerves and chiasm superiorly. The mass was subtotally resected. No attachment of the mass to brain stem or cerebellar structures was noted. Histological examination identified the tissue as developing cerebellum. The cytoarchitecture and cellular constituents of the cerebellar tissue were only slightly distorted. All cerebellar cortical constituents were arranged anatomically, and an external granular cell layer was present superficially. The latter was actively proliferating and appropriately cellular for the infant's age. The clinical presentation of ectopic cerebellum as a suprasellar mass in an infant is highly unusual. Moreover, this example illustrates the ability of cerebellar tissue to mature appropriately in a site distant from the posterior fossa, removed from ascending and descending afferent projections. Intrinsic signaling mechanisms appear sufficient to direct histogenesis in developing cerebellar cortex.


Author(s):  
Gowri Sankar M. ◽  
Somanath B. M. ◽  
Raveendra P. Gadag ◽  
Manu C. B. ◽  
Nagaraj M.

<p>Osteoma is a benign slow growing bone tumour with predominance in long bones, and is rare in the skull. They are a rare occurrence in the mastoid temporal bone, being usually asymptomatic and treated mainly for cosmetic reasons. We report a case of a young female who presented with a history of swelling in the left post aural region since 10 years. Swelling was gradually increasing in size and was painless. Patient underwent excision of the tumour without any significant complication. As per our knowledge this appears to be the largest tumour reported in literature. The clinical presentation and radiological features of osteoma are characteristic but differential diagnosis should include eosinophilic granuloma, giant cell tumour, monostotic fibrous dysplasia, solitary variant of multiple osteoma, and osteoblastic metastasis. Osteomas present on the mastoid or squamous portion of the temporal bone need to be dealt for cosmetic purposes or if they are causing symptoms, while surgery should include careful removal of periosteal cover and safe margin of the mastoid cortex around it. </p>


2020 ◽  
Vol 24 (2) ◽  
pp. 103-106
Author(s):  
Jeong Hwan Choi

Granular cell tumor (GCT) is a rare, benign neoplasm of Schwann cell origin. GCT is composed of cells with eosinophilic granular cytoplasm. GCT presents as a solitary painless nodule. Because of their subtle clinical presentation, GCTs are often misdiagnosed. This report of a 47-year-old woman with an auricular GCT serves to highlight that complete excision and histopathological evaluation should be attempted even in apparently benign cases, to ensure complete cure.


2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
Dustin Taylor ◽  
Natalie Kash ◽  
Sirunya Silapunt

Lymphangioma circumscriptum is a superficially localized variant of lymphangioma. The characteristic clinical presentation is a “frogspawn” grouping of vesicles or papulovesicles on the proximal limb or limb girdle areas. Though most lymphangiomas develop congenitally, the lymphangioma circumscriptum subtype is known to present in adults. We report a case of lymphangioma circumscriptum on the left inframammary area of an African American female with an unusual supernumerary nipple-like clinical presentation. Our patient presented with a firm, smooth, hypopigmented papule, and the clinical diagnosis of keloid was made initially. However, she returned reporting growth of the lesion and was noted to have a firm, exophytic, lobulated, pink to skin-colored nodule. Histopathological examination demonstrated dilated lymphatic vessels, consistent with the diagnosis of lymphangioma. The presentation as a firm, hypopigmented papule and later exophytic, lobulated, skin-colored nodule in our case represents a clinical presentation of lymphangioma circumscriptum not previously described in the literature. Correct diagnosis in lymphangioma circumscriptum is vital, as recurrence following surgical resection and secondary development of lymphangiosarcoma and squamous cell carcinoma following treatment with radiation have been reported. Thus, it is important to consider lymphangioma circumscriptum in the differential of similar lesions in the future to allow appropriate diagnosis, treatment, and monitoring.


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