scholarly journals Multiple Koenen Tumors, a Rare Entity: Combination Treatment with 1% Topical Sirolimus Electrofulguration and Excision

2020 ◽  
Vol 7 (1) ◽  
pp. 66-70
Author(s):  
Vishalakshi Viswanath ◽  
Jay D. Gupte ◽  
Niharika Prabhu ◽  
Nilima L. Gour
Keyword(s):  
Tuberous Sclerosis ◽  
Combination Treatment ◽  
Tumor Regression ◽  
Surgical Excision ◽  
Tumor Formation ◽  
Rare Entity ◽  
Rare Presentation ◽  
Cutaneous Manifestations ◽  
Elderly Female

<b><i>Introduction:</i></b> Koenen tumors are benign, cutaneous manifestations of tuberous sclerosis. These are disfiguring, painful, and challenging to treat as they frequently recur. We report a case of long-standing, multiple Koenen tumors affecting all twenty nails in an elderly female who was successfully treated with a combination of topical sirolimus 1%, surgical excision, and electrofulguration. <b><i>Case Report:</i></b> A 57-year-old lady presented with multiple, asymptomatic periungual, and subungual tumors affecting all twenty nails since 27 years. Cutaneous examination revealed confetti macules, ash-leaf macule, and shagreen patch over trunk. Nail biopsy was compatible with Koenen’s tumor. Computerized tomography of brain showed diffuse patchy sclerosis. The tumors were treated with topical sirolimus 1% ointment for 10 months with excellent regression. Electro­fulguration for both great toenails and surgical excision of right thumbnail periungual fibroma was done. 1% sirolimus was advised after the surgical treatment. There were no adverse effects or recurrence of tumors over a 2-year follow-up. <b><i>Discussion:</i></b> Topical sirolimus 1% was effective in tumor regression and preventing new tumor formation. Larger tumors that interfered in daily chores were treated with excision and electrofulguration. Thus, a combination treatment for this rare presentation of tuberous sclerosis provided optimum results.

Adrenal pheochromocytoma with involvement of aorta and left renal artery: salvaged with adrenalectomy and nephrectomy

2021 ◽  
Vol 14 (9) ◽  
pp. e244297
Author(s):  
Shekhar Sathaye ◽  
Kalpesh Mahesh Parmar ◽  
Santosh Kumar ◽  
Pulkit Rastogi
Keyword(s):  
Renal Artery ◽  
Renal Involvement ◽  
Renal Tissue ◽  
Preoperative Imaging ◽  
Beta Blockade ◽  
Left Renal Artery ◽  
Rare Entity ◽  
Rare Presentation ◽  
Adrenal Pheochromocytoma

Large adrenal pheochromocytomas encasing the renal artery are a rare entity. The management of such challenging cases is surgical resection. The involvement of renal tissue and renal artery may necessitate meticulous dissection and concomitant nephrectomy. Here, we present a case of 41-year-old man diagnosed with left adrenal pheochromocytoma with complete encasement of left renal artery and partial encasement of aorta. Open left adrenalectomy and nephrectomy was performed after adequate preoperative optimisation. The patient is doing well at 6-month follow-up. Large adrenal pheochromocytoma with renal involvement is a rare presentation and requires optimal preoperative imaging, adequate preoperative alpha and beta blockade and meticulous surgical technique.

scholarly journals Umbilical endometriosis along with peritoneal endometriosis: a case report

2019 ◽  
Vol 8 (5) ◽  
pp. 2106
Author(s):  
T. Ramani Devi ◽  
C. Archana Devi ◽  
C. Aparna Devi
Keyword(s):  
Surgical Excision ◽  
Reproductive Age ◽  
Rare Entity ◽  
Pelvic Endometriosis ◽  
The Past ◽  
Peritoneal Endometriosis ◽  
Laparoscopic Sterilization ◽  
Reproductive Age Group ◽  
Umbilical Endometriosis

Incidence of endometriosis is around 10 to 15% in women of reproductive age group. Umbilical endometriosis is a very rare entity. Extra genital endometriosis accounts to 3% of endometriosis. Incidence of umbilical endometriosis is 0.5%-4% of extra genital endometriosis. 30 years old multi gravida was referred to our hospital with c/o periodic bleeding from the umbilicus for the past 3 months. She was also having dysmenorrhoea for about 3 months. On examination, patient had a small bluish nodule in the umbilicus around 1.5x1.2 cm in size. Clinically there was suspicion of pelvic endometriosis as the uterus was retroverted and fixed. CT abdomen showed a small hypo-echoeic area in the umbilicus and uterus was adenomyotic with normal ovaries. Patient was given the option of laparoscopy and excision of umbilicus, as there was suspicion of peritoneal endometriosis and the patient also insisted upon laparoscopic sterilization. Laparoscopy showed early peritoneal endometriosis with pelvic adhesions and the same adhesiolysis was done along with cauterization of endometriosis. Sterilization was also done as per the patient’s request. Umbilical excision and layer closure was done. Umbilical endometriosis is a rare entity. This patient had associated early pelvic endometriosis. Umbilical endometriosis could be secondary to the lympho vascular spread from the pelvic endometriosis or primary umbilical endometriosis. History, clinical and imaging were pointing towards umbilical endometriosis. Surgical excision of umbilical endometriosis and cauterisation of early pelvic endometriosis were done. Patient needs follow up. Umbilical endometriosis may be primary or secondary which needs total excision and follow up.

scholarly journals Scar endometriosis-rare and painful entity: a case report

2021 ◽  
Vol 10 (5) ◽  
pp. 2104
Author(s):  
Anita Yadav ◽  
Jyoti Baghel ◽  
Rajneesh Rawat ◽  
Avinash Prakash
Keyword(s):  
Rare Case ◽  
Rectus Sheath ◽  
Surgical Excision ◽  
Tertiary Level ◽  
Rare Entity ◽  
Pelvic Endometriosis ◽  
Rare Type ◽  
Detailed History ◽  
Scar Endometriosis

Scar endometriosis is a rare type of extra-pelvic endometriosis that develops following obstetrical and gynecological surgeries. It is a rare entity, though probably on the rise, due to the increase in caesarean sections performed worldwide. This manuscript reports a rare case of scar endometriosis involving rectus sheath following repeat caesarean section and was managed at a tertiary level centre. The patient required surgical excision of the lesion and was kept on regular follow-up following surgery. The physiopathology of scar endometriosis is complex; its symptomatology is rich and diverse but detailed history, thorough clinical examination along with imaging and histopathological evaluation is usually efficient in diagnosing the condition.

scholarly journals A Rare Presentation of Schwannoma as a Purely-Cystic Superficial-Intermuscular Extra-Spinal Swelling over Upper Back.

2020 ◽  
Author(s):  
Srinjoy Saha
Keyword(s):  
Surgical Excision ◽  
Thoracic Vertebrae ◽  
Rare Presentation ◽  
Complete Surgical Excision ◽  
Rare Tumours ◽  
S 100 ◽  
One Year ◽  
Atypical Presentations ◽  
Upper Thoracic

Abstract Schwannomas are rare tumours arising from peripheral nerve sheath. Here, a 44-year-old lady came to our clinic with an asymptomatic progressively-enlarging swelling over her upper back. It was fluctuant, with an absence of pain and tenderness. MRI showed a benign, purely-cystic, superficial-intermuscular, extra-spinal swelling nearby upper thoracic vertebrae. Complete surgical excision proceeded smoothly through a well-defined plane between the swelling and the muscles. It was not attached to any identifiable nerve. A 6.5x5.0x2.5 cm ovoid lesion with a glistening whitish-grey capsule was excised and wound reconstructed in layers. Histopathology showed hypercellular areas with nuclear palisading or oval-shaped Verocay bodies. Only S-100 tested positive amongst the five-antigen immunohistochemistry, thus establishing the diagnosis of a schwannoma. Postoperatively, a one-year follow-up period was uneventful. Schwannomas can surprise clinicians by arising anywhere and with atypical presentations. It needs to be in the differential diagnoses of any asymptomatic slowly-growing lesion.

scholarly journals Diagnosis and Treatment of Tuberous Sclerosis Manifestations in Children: A Multicenter Study

2018 ◽  
Vol 49 (03) ◽  
pp. 193-199 ◽  
Author(s):  
Daniel Ebrahimi-Fakhari ◽  
Ludwig Gortner ◽  
Martin Poryo ◽  
Michael Zemlin ◽  
Alfons Macaya-Ruiz ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Clinical Symptoms ◽  
Medical Center ◽  
Interquartile Range ◽  
University Hospital ◽  
Cardiac Rhabdomyoma ◽  
Cutaneous Manifestations ◽  
Ocular Manifestations ◽  
Made In

AbstractTuberous sclerosis complex (TSC) is a genetic disease with a significant morbidity and mortality. We conducted a retrospective analysis of two cohorts (Vall d'Hebron University Hospital [HVH], Barcelona, Spain, 1982–2015, and at Saarland University Medical Center [UKS], Homburg, Germany, 1998–2015) to assess prevalence and treatment of TSC associated manifestations and to evaluate if the follow-up was in line with published recommendations. This was considered if more than 15% of patients did not receive adequate examination with regard to potential organ involvement. A definite diagnosis was made in 52 patients (96%), and a possible diagnosis was made in 2 patients (4%). Thirty-four (63%) patients were from HVH and 20 (37%) from UKS. Median age at first presentation was 6 months (interquartile range: 0–38 months), and median time of follow-up was 6 years (interquartile range: 2–13 years). Clinical symptoms that led to a diagnosis of TSC were cardiac rhabdomyoma (22/54), epilepsy (20/54), and cutaneous manifestations (4/54). Assessment of neuropsychiatric, renal, and ocular manifestations was inadequate in both hospitals, whereas cutaneous manifestation was inadequate at UKS only. Our data demonstrate insufficient examinations in a substantial number of TSC patients with regard to neuropsychiatric, renal, ocular, and cutaneous manifestations. The recently published guidelines may prove valuable in establishing a more comprehensive approach.

scholarly journals Desmoplastic Fibroma Recurrence Associated with Tuberous Sclerosis in a Young Patient

2018 ◽  
Vol 2018 ◽  
pp. 1-4
Author(s):  
A. M. Espinoza-Coronado ◽  
J. P. Loyola-Rodríguez ◽  
J. H. Olvera-Delgado ◽  
J. O. García-Cortes ◽  
J. F. Reyes-Macías
Keyword(s):  
Tuberous Sclerosis ◽  
Surgical Excision ◽  
Clinical Findings ◽  
Desmoplastic Fibroma ◽  
Volume Increase ◽  
Benign Pathology ◽  
The Brain ◽  
Recurrence Frequency ◽  
Hard Consistency

Case Report. A nine-year-old patient with a diagnosis of tuberous sclerosis (with no pathological record) that showed calcifications at the brain level. Besides, the case showed the Vogt triad (epilepsy, mental retardation, and sebaceous adenoma). The patient clinically showed a volume increase of hard consistency, without suppuration and no sessile that included the following teeth 73, 74, and 75. Cone beam computed tomography (CBCT) was obtained, and it displayed a delimited unilocular lesion. After surgical excision, the histopathological report was desmoplastic fibroma (DF). It was observed that the patient had an aggressive recurrence of DF at four months after surgery treatment. Due to these clinical findings, resective osseous surgery and curettage were carried out. It is uncommon to find these two pathologies together (DF and tuberous sclerosis). Since DF is a benign pathology but very invasive and destructive, it is necessary a constant follow-up examination due to a high recurrence frequency.

scholarly journals Low grade fibromyxoid sarcoma of the perineum: a surgical challenge

2016 ◽  
Vol 4 (1) ◽  
pp. 429
Author(s):  
Afzal Anees ◽  
Kaushal D. Singh ◽  
Shehtaj Khan
Keyword(s):  
Local Recurrence ◽  
Surgical Management ◽  
Surgical Excision ◽  
Low Grade ◽  
Rare Presentation ◽  
Desmoid Fibromatosis ◽  
Fibromyxoid Sarcoma ◽  
Slow Growing ◽  
Late Metastasis

Low-grade fibromyxoid sarcoma is a rare, slow growing and deceptively benign-appearing neoplasm. It is presently diagnosed on the basis of histopathology (typical fibro-myxoid appearance), immunohistochemistry (vimentin staining) and cytogenetics [chimeric FUS/CREB3L2 gene produced by t (7;16), (q33;p11)]. This tumour should be differentiated from other resembling tumours like myxofibrosarcoma, sclerosing epitheloid fibrosarcoma, desmoid fibromatosis and others. It has the potential for local recurrence and late metastasis, if not treated adequately. Although surgical excision is the only hope for treatment, reporting of details of surgical management have often been neglected for this primarily pathological entity. We report a case of a 30-year female who had a perineal mass which was managed by wide surgical excision. It was diagnosed as low-grade fibromyxoid sarcoma on the basis of histopathology and immunohistochemistry. This extremely rare presentation is, thus, discussed in context of challenging nature of its surgical excision. Patient is in follow-up and has no recurrence even after 5 years.

Delayed metastasis in patients with intraocular retinoblastoma: A review of three cases

2020 ◽  
pp. 112067212094628
Author(s):  
Vijitha S Vempuluru ◽  
Saumya Jakati ◽  
Swathi Kaliki
Keyword(s):  
Metastatic Disease ◽  
Tumor Regression ◽  
External Beam Radiotherapy ◽  
Case Series ◽  
Surgical Excision ◽  
Primary Treatment ◽  
Retrospective Case ◽  
Temporal Fossa ◽  
Intravitreal Chemotherapy

Purpose: To discuss the clinical presentation, management, and outcome of delayed metastasis in retinoblastoma (RB). Methods: Retrospective case series of three patients. Results: Mean age at diagnosis of RB was 29 months (median, 28 months; range, 11–48 months). All were males with non-familial bilateral intraocular RB. Primary treatment for RB included intravenous chemotherapy in all three cases. Secondary treatment included transpupillary thermotherapy/cryotherapy ( n = 6 eyes), periocular chemotherapy ( n = 2 eyes), intravitreal chemotherapy ( n = 1 eye), intra-arterial chemotherapy ( n = 1 eye), external beam radiotherapy (EBRT; n = 2 eyes), and enucleation ( n = 2 eyes). Primary tumor regression was achieved in all cases and remained status quo at the time of diagnosis of distant metastasis. Two patients developed bone metastasis (ulna; tibia) and one developed soft tissue metastasis (temporal fossa) over a mean follow-up period of 6 years (median, 7 years; range, 5–8 years) from diagnosis of RB. Mean age of detection of metastatic disease was 8 years (median, 8 years; range, 7–9 years). All the lesions were solitary and the diagnosis of metastatic retinoblastoma was confirmed by tissue biopsy. Metastatic disease was treated with surgical excision ( n = 1), chemotherapy ( n = 2), and EBRT ( n = 2). All patients are alive, with two patients free of disease over a mean follow-up period of 23 months (median, 23 months; range, 12–33 months); and 1 in remission 7 months after completion of EBRT. Conclusion: Long-term follow-up of RB cases is mandatory. In spite of intraocular tumor regression, metastasis can still occur many years after treatment of RB.

Inflammatory myofibroblastic lung tumor with dysphagia in a 4-year-old child

2017 ◽  
Vol 25 (9) ◽  
pp. 653-655 ◽  
Author(s):  
Vinitha V Nair ◽  
Kunal Krishna Singh ◽  
Joseph Thomas Kathayanat ◽  
Ratish Radhakrishnan ◽  
Akash Babu ◽  
...  
Keyword(s):  
Weight Loss ◽  
Respiratory Symptoms ◽  
Inflammatory Myofibroblastic Tumor ◽  
Lung Tumor ◽  
Gastroesophageal Junction ◽  
Surgical Excision ◽  
Pulmonary Vasculature ◽  
Rare Presentation ◽  
Inflammatory Myofibroblastic Tumors

Inflammatory myofibroblastic tumors of the lung are rare in adults but common among pediatric lung tumors. A 4-year-old girl was evaluated for respiratory symptoms, dysphagia, and weight loss. Radiology revealed a right hilar mass that persisted despite antibiotic treatment. On exploration, a hilar mass involving the pulmonary vasculature, diaphragm and gastroesophageal junction was found. Aggressive surgical excision including a right pneumonectomy was performed. Histopathology confirmed an inflammatory myofibroblastic tumor with no features of malignancy. At the 2-year follow-up, the child was clinically stable with no recurrence. Dysphagia is a very rare presentation of such tumors.

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