Lipomatous hypertrophy of the interventricular septum.

BACKGROUND AND AIMS: Lipomatous hypertrophy of the interatrial septum is an uncommon type of cardiac adipose tumor. It is extremely rare for these lesions to be located in the interventricular septum. METHODS: We report this case of incidentally diagnosed lipomatous hypertrophy of the interventricular septum with right ventricular outflow obstruction which caused minimal symptoms and Wolff-Parkinson-White pattern on EKG. CONCLUSION: We also discuss the diagnosis and management of this condition.

Intraoperative optical coherence tomography for soft tissue sarcoma differentiation and margin identification.

BACKGROUND AND OBJECTIVE: Sarcomas are rare but highly aggressive tumors, and local recurrence after surgical excision can occur in up to 50% cases. Therefore, there is a strong clinical need for accurate tissue differentiation and margin assessment to reduce incomplete resection and local recurrence. The purpose of this study was to investigate the use of optical coherence tomography (OCT) and a novel image texture-based processing algorithm to differentiate sarcoma from muscle and adipose tissue. STUDY DESIGN AND METHODS: In this study, tumor margin delineation in 19 feline and canine veterinary patients was achieved with intraoperative OCT to help validate tumor resection. While differentiation of lower-scattering adipose tissue from higher-scattering muscle and tumor tissue was relatively straightforward, it was more challenging to distinguish between dense highly scattering muscle and tumor tissue types based on scattering intensity and microstructural features alone. To improve tissue-type differentiation in a more objective and automated manner, three descriptive statistical metrics, namely the coefficient of variation (CV), standard deviation (STD), and Range, were implemented in a custom algorithm applied to the OCT images. RESULTS: Over 22,800 OCT images were collected intraoperatively from over 38 sites on 19 ex vivo tissue specimens removed during sarcoma surgeries. Following the generation of an initial set of OCT images correlated with standard hematoxylin and eosin-stained histopathology, over 760 images were subsequently used for automated analysis. Using texture-based image processing metrics, OCT images of sarcoma, muscle, and adipose tissue were all found to be statistically different from one another (P ≤ 0.001). CONCLUSION: These results demonstrate the potential of using intraoperative OCT, along with an automated tissue differentiation algorithm, as a guidance tool for soft tissue sarcoma margin delineation in the operating room. Lasers Surg. Med. 49:240-248, 2017. © 2017 Wiley Periodicals, Inc.

Lipomatous tumours of the head and neck: a spectrum of biological behaviour.

Lipomatous tumours are rare in the head and neck region. Their biological behaviour varies greatly, from absolutely benign to histologically benign but locally infiltrative and, finally, invasive with metastatic potential. Each lipomatous tumour has to be treated accordingly. It is of paramount importance before eventual surgery is planned to perform adequate imaging, diagnostic biopsy and careful assessment. Only in small (<5 cm), superficial soft tissue tumours or when magnetic resonance imaging has demonstrated specific features of lipoma, may diagnostic biopsy be omitted. In these cases, expectant management or simple excision is appropriate. Adequate preoperative diagnosis is important to assure adequate tumour control as well as optimal functional and cosmetic outcome. The major problem in the treatment of lipomatous tumours of the head and neck region is the presence of nearby delicate structures. Especially, wide surgical excision of liposarcomas may be hindered by anatomic constraints and may result in impaired functional and cosmetic outcome. Neoadjuvant radiotherapy and specific systemic chemotherapy may be helpful in the treatment of liposarcoma, especially when unresectable or when primary surgery is expected to result in poor oncological, functional or cosmetic outcome. Greater emphasis placed on the underlying biology of individual sarcoma subtypes, development and evaluation of novel therapies and greater specificity in the selection of chemotherapy agents based on activity in individual histological subtypes are expected to lead to improved efficacy of systemic treatment.

[A case of late perirenal fat recurrence after partial nephrectomy for T1A renal cell carcinoma].

An 84-year-old man had undergone laparoscopic partial nephrectomy for right renal cell carcinoma (RCC), cT1aN0M0 in 2003. The histopathological diagnosis was clear cell carcinoma, grade 1, v (-), surgical margin negative, pT1a. Nine years and 10 months postoperatively, computed tomography scans demonstrated tumors on right renal fossa. As we could not detect other metastatic lesions, we diagnosed him with local recurrence of RCC and planned the surgery with curative intent. He underwent laparoscopic resection of retroperitoneal tumors. The histopathological diagnosis was clear cell carcinoma, grade 2 ＞ 3, v (-), surgical margin negative, and confirmed recurrence of RCC. In retrospective review of 176 cases of pT1a renal cell carcinoma with partial nephrectomy in our institute, 3 patients (1.7%) developed local recurrence and 2 patients (1.1%) developed late local recurrence.

Large parasitic myomas in abdominal subcutaneous adipose tissue along a previous myomectomy scar.

The incidence of iatrogenic parasitic uterine myomas associated with the use of a laparoscopic morcellator has been increasing over the past decade. Recently, we encountered a very rare case with a large parasitic myoma measuring 12 cm in diameter in the abdominal subcutaneous adipose tissue along an abdominal longitudinal surgical scar. The patient had twice undergone abdominal myomectomy for multiple fundal myomas. This is the first report describing a case with as large a parasitic myoma presenting in the suprafascial adipose tissue under the surgical scar after laparotomy. In such a case demonstrating a solid tumor of unknown cause after a gynecologic surgical procedure, a parasitic myoma must be included in the differential diagnosis.

Surgical technique of the transoral approach to remove a lipoma of the buccal fat pad.

BACKGROUND: In 1727, Heister (Compendium anatomicum. Altdorf, Guill, Koleshii: editio tertia 1727: 134, table VIII and figs. 36-37) described the buccal fat pad (BFP) as an independent anatomic structure of the face; in 1801, Bichat (Anatomie generale appliquee a la physiologie et a la medecine. Paris, France: Brosson, Gabon et Cie Libraires, 1801:60) reported his fatty histologic finding. According to the literature, several pathologic tumorous conditions can arise from BFP, such as lipoma, lipoblastomatosis, liposarcoma, hemangioma, arteriovenous malformation, and nodular fasciitis; all of which are rare. After a revision of the English literature performed through PubMed between 1948 and 2008, we found 10 cases of lipomas arising from the BFP (7 cases are simple subtype, 2 are spindle cell lipoma, and 1 is fibrolipoma). The aims of this study were to introduce our clinical report of this rare pathologic entity, describe the surgical technique of the transoral approach, and discuss the potential pitfalls regarding the preoperative diagnosis and the close interrelation among the BFP, the facial buccal branches (FBBs), and the parotid duct (PD). CLINICAL REPORT: A 43-year-old man was referred to the Maxillofacial Unit of the Novara Major Hospital with a 6-month history of a painless swelling in the right cheek. Clinical examination revealed a clearly visible, tender, slightly fluctuant mass, situated anterior to the masseter muscle and extended to the submandibular region. The patient underwent an ultrasound, a computed tomography, and a magnetic resonance imaging. Under general anesthesia with nasotracheal intubation, the patient underwent intraoral resection of BFP lipoma. DISCUSSION: The 2 major areas of discussion are the potential pitfall regarding the preoperative diagnosis and the close anatomic interrelation among the BFP, the FBB, and the PD. First, the spindle cell lipoma, one of the most common BFP lipoma variant, can be histologically and clinically similar to a well-differentiated liposarcoma, which can be recurrent and metastatic. This issue warrants that a careful workup of the tumorous mass of the buccal space and a BFP origin must be considered in every situation. Finally, according to the recent literature, the anatomic variations of the interrelation between the FBB and the BFP are classified into 2 groups: (1) FBB passing lateral to the BFP and (2) branches crossing inside the BFP. The anatomic variations of the interrelation between the PD and the BFP are classified into 3 groups: (1) PD passing lateral to the BFP, (2) PD crossing deep to the BFP, and (3) PD running along the superior border of the BFP.

[Lipid cell variant urothelial carcinoma of the renal pelvis. A case report and literature review]. / Carcinoma urotelial lipoideo de pelvis renal. Aportación de un nuevo caso y revisión de la literatura de esta variante de carcinoma urotelial.

There are many variants of urothelial carcinoma. One of the most infrequent is formed by cells with a lipid content and an adipose tissue appearance. Only 43 cases have been reported in the bladder, 2 in the renal pelvis and 1 case in the ureter. We present a third case in the renal pelvis; the patient is alive and free of disease 103 months post diagnosis.

Surgical management of truncal and extremities atypical lipomatous tumors/well-differentiated liposarcoma: A systematic review of the literature.

INTRODUCTION: Atypical lipomatous tumors or well-differentiated liposarcomas (ALT/WDLS) are low-grade soft tissue tumors that are commonly located on the trunk and extremities. There is no consensus on the best surgical approach for ALT/WDLS. METHODS: A systematic literature review of PubMed, Medline, Embase, Scopus, and google scholar was performed. All published studies on trunk or extremities ALT/WDLS with reported outcome data were considered and independently screened for inclusion by at least two of the authors. RESULTS: A total of eighteen studies comprising 793 patients with ALT/WDLS were included. 580 patients underwent marginal excision, with local recurrence observed in 69 (11.9%). 213 patients underwent wide excision with local recurrence in 7(3.3%). Recurrent tumors were successfully re-resected with marginal or wide excision. Dedifferentiation was confirmed in 9 patients (1.1%), and a distant pulmonary metastasis in 1 patient (0.1%). DISCUSSION: Marginal excision of truncal or extremities ALT/WDLS results in a slightly higher local recurrence rate. However, recurrences are almost always amenable to re-resection. The findings support the use of marginal excision for truncal or extremities ALT/WDLS.

A Diagnostic Dilemma of a Subcutaneous Hibernoma: Case Report.

BACKGROUND Subcutaneous lipomatous lesions are commonly encountered in clinical practice. Hibernoma is a rare subtype of the benign lipomatous tumor, representing 1% of all types. It poses a challenge due to the difficulty of differentiating it from atypical lipomatous lesions and liposarcomas, which may lead to possible inappropriate diagnosis and management. CASE REPORT We report a case of a 33-year-old male who presented with a right upper thigh swelling noticed some time prior to presentation that had started increasing in size prior to his presentation. The magnetic resonance imaging (MRI) was unable to rule out atypical lipomatous tumor and liposarcoma. An ultrasound-guided biopsy gave a diagnosis of hibernoma. The patient underwent a wide local excision, which confirmed the diagnosis of hibernoma. At the 3-year follow-up, there was no evidence of local recurrence. CONCLUSIONS Hibernoma has been reported in the literature to be discovered incidentally by radiological imaging done for other causes. However, hibernomas raise a diagnostic challenge because in most imaging modalities they are indistinguishable from other malignant tumors. A wide local excision with negative margins is key to resolving the diagnostic dilemma that a hibernoma presents, as it will provide a definitive diagnosis differentiating it from other lipomatous lesions and prevent any future recurrence. Caution is advised when dealing with lipomatous lesions, as they often overlap with malignancy. Furthermore, an MRI should be done for any subcutaneous lesion that is larger than 5 cm or shows recent growth. A biopsy can resolve the diagnostic dilemma with caution to the hypervascularity of such tumors.

Lipoblastoma--an unfamiliar but important diagnosis. A case series and literature review.

Lipoblastoma and its infiltrative variant lipoblastomatosis are rare adipose tissue tumours seen in infants and children. Many surgeons are unfamiliar with these uncommon lesions and hence they are suboptimally treated. We report a case series of six patients in our tertiary paediatric hospital. Cases were reviewed retrospectively with reference to demographics, investigations, diagnosis and their management. Lipoblastomas are easily misdiagnosed and excision before proper investigations may result in incomplete resection, recurrence and further potentially mutilating surgery.

Omental lipoblastoma.

A large intra-abdominal mass was discovered in a 6-month-old boy during a routine well-child examination. Imaging studies revealed a solid mass which appeared to arise from the left lobe of the liver, extending caudally and filling the entire pelvis. At the time of surgical excision, the mass was found to be unassociated with the liver, but was instead localized to the omentum. Histologic examination revealed adipocytes of varying stages of maturation arranged in a lobular architecture, consistent with a lipoblastoma. This unusual tumor is only the eighth reported omental lipoblastoma [J. Hicks, A. Dilley, D. Patel, J. Barrish, S. Zhu, M. Brandt, Lipoblastoma and lipoblastomatosis in infancy and childhood: histologic, ultrastructural, and cytogenetic features. Ultrastruct. Pathol. 25 (2001) 321-333; J. Harrer, G. Hammon, T. Wagner, M. Bolkenius, Lipoblastoma and lipoblastomatosis: a report of two cases and review of the literature. Eur. J. Pediatr. Surg. 11 (2001) 342-349; S. Weiss and J. Goldblum, Enzinger and Weiss's Soft Tissue Tumors, fourth ed., Mosby, St. Louis, MO, 2001, pp. 601-605, 670-686; S. Soin, S. Andronikou, R. Lisle, K. Platt, K. Lakhoo, Omental lipoblastoma in a child; diagnosis based in CT density measurements. J. Pediatr. Hematol. Oncol. 28(1) (2006) 57-58; A. Prando, S. Wallace, J.L. Marins, R.M. Pereira, E.R. de Oliveira, M. Alvarenga, Sonographic features of benign intraperitoneal lipomatous tumors in children-report of 4 cases. Pediatr. Radiol. 20(8) (1990) 571-574; C. Blank, E. Schoenmakers, P. Rogalla, E. Huys, A. Van Rijk, N. Drieschner, J. Bullerdiek, Intragenic breakpoint within RAD51L1 in a t(6;14)(p21.3;q24) of a pulmonary chondroid hamartoma. Cytogenet. Cell Genet. 95 (2001) 17-19; S. Ingraham, R. Lynch, S. Kathiresan, A. Buckler, A. Menon, hREC2, a RAD51-like gene, is disrupted by t(12;14)(q15;q24.1) in a uterine leiomyoma. Cancer Genet. Cytogenet. 115 (1999) 56-61]. Cytogenetics revealed a karyotype of 46,XY,t(8;14)(q13;q24). While lipoblastomas characteristically involve 8q, only one prior case has been reported with 14q24 as its fusion partner [M. He, K. Das, M. Blacksin, J. Benevenia, M. Hameed, A translocation involving the placental growth factor gene is identified in an epithelioid hemangioendothelioma. Cancer Genet. Cytogenet. 168 (2006) 150-154]. We report this unique case of an omental lipoblastoma with a focus on its unusual karyotype, as well as its differentiation from myxoid liposarcoma.

Osteolipoma of the eyelid.

A 50-year-old woman presented with a nodular swelling on her right upper eyelid. The tumour was diagnosed as osteolipoma histologically after resection. Osteolipoma is a very rare variant of lipoma and only a few cases affecting the head and neck are reported in literature. To the best of the author's knowledge this is the first reported case of osteolipoma of the eyelid.

Lipoblastoma in children: an analysis of 5 cases.

Lipoblastoma is a very rare benign tumour that is caused by embryonal fat. The present five cases of lipoblastoma operated on during the years 1996-2005. The localization of the lipoblastomas in our series were very unusual. A six-monthold girl with giant mediastinal lipoblastoma; a two-year old boy with very rare lipoblastoma of the kidney; a three-year old boy with mesenterial lipoblastoma; a seven-year old boy with mesenterial lipoblastoma; and an eight-week old girl with perineal localization. Histological diagnosis can be difficult. The basic differential diagnosis is to be made between lipoblastoma, myxoid, and round cell liposarcoma. In our sample group of patients all lipoblastomas were successfully and completely removed and we did not see any recurrence of the tumours. In only one case was more radical surgery needed. One patient with mesenterial lipoblastoma had to undergo a 30 cm long resection of the small intestine.

Extranodal extension of lymph node metastasis influences recurrence in prostate cancer: a systematic review and meta-analysis.

The extranodal extension (ENE) of nodal metastasis involves the extension of neoplastic cells through the lymph node capsule into the perinodal adipose tissue. This morphological feature has recently been indicated as an important prognostic factor in various cancer types, but its role in prostate cancer is still unclear. We aimed to clarify it, performing the first meta-analysis on this issue, comparing prognostic parameters in surgically treated, node-positive prostate cancer patients with (ENE+) vs. without (ENE-) ENE. Data were summarized using risk ratios (RRs) for number of deaths/recurrences and hazard ratios (HRs), with 95% confidence intervals (CI), for the time-dependent risk related to ENE positivity. Six studies followed-up 1,113 patients with N1 prostate cancer (658 ENE+ vs. 455 ENE-) for a median of 83 months. The presence of ENE was associated with a significantly higher risk of biochemical recurrence (RR = 1.15; 95%CI: 1.03-1.28; I2 = 0%; HR = 1.40, 95%CI: 1.12-1.74; I2 = 0%) and "global" (biochemical recurrence and distant metastasis) recurrence (RR = 1.15; 95%CI: 1.04-1.28; I2 = 0%; HR = 1.41, 95%CI: 1.14-1.74; I2 = 0%). ENE emerged as a potential prognostic moderator, earmarking a subgroup of patients at higher risk of recurrence. It may be considered for the prognostic stratification of metastatic patients. New possible therapeutic approaches may explore more in depth this prognostic parameter.

Oncogenic osteomalacia: localization of underlying peripheral mesenchymal tumors with use of Tc 99m sestamibi scintigraphy.

OBJECTIVE: To highlight a strategy for potential detection of mesenchymal tumors in oncogenic malacia, as illustrated by 3 cases. METHODS: Three case reports are presented in which successful localization of the offending neoplasm was accomplished by using whole-body Tc 99m sestamibi scanning. Alternative localization techniques are also reviewed. RESULTS: Oncogenic osteomalacia occurs infrequently and is caused by neoplasms that secrete phosphatonins, substances that interfere with proximal tubular resorption of phosphorus and can result in phosphaturia, hypophosphatemia, reduced 1,25-dihydroxyvitamin D concentration, and osteomalacia. Removal of the underlying neoplasm results in complete resolution of all biochemical, pathologic, and physical manifestations of this disorder, as shown in our 3 patients. Because the neoplasms are small and can occur in any tissue compartment, they are difficult to localize, a feature that often results in therapeutic failure. CONCLUSION: We conclude that use of whole-body Tc 99m sestamibi scanning may be an appropriate and cost-effective initial strategy for the localization of peripheral phosphatonin-secreting tumors.

Juxtaarticular Myxoma in a Pigtail Macaque (Macaca nemestrina).

A 10-y-old pigtail macaque presented with a subcutaneous, soft-tissue mass overlying the right stifle joint. Here we describe the clinical case and histopathologic and immunohistochemical analysis of this lesion. This case represents the first published report of juxtaarticular myxoma in a pigtail macaque.

Lipoblastoma of infancy mimicking hemangioma of infancy.

Lipoblastomas are rare benign tumors of infancy that usually affect children younger than 3 years. Most lipoblastomas (70%) occur on the extremities. Lipoblastomas may mimic other infantile tumors, including hemangiomas, hibernomas, lipomas, and liposarcomas, and correct diagnosis is necessary to ensure appropriate treatment. Lipoblastomas fall under 2 discrete subtypes: well-circumscribed lipoblastomas and diffuse lipoblastomatosis. Both types present with firm, nontender masses of lobulated, well-circumscribed soft tissue. Histologically they can be highly vascularized with plexiform capillaries, often with an individual feeder artery to each lobule. Complete surgical removal is the recommended treatment. Only 2 cases of lipoblastomas of the cheek have been reported in the English-language literature. We present the case of a young child with a cheek lipoblastoma, emphasizing the importance of correct diagnosis and highlighting techniques used to provide suitable treatment.

Recurrent maturing perineal lipoblastoma.

Lipoblastoma is a rare benign neoplasm of fetal adipose tissue that we see mostly in infants and young children less than 3 years of age. Most lipoblastomas occur on the extremities, trunk, head and neck, and various other organs have been described. We report a case of a recurrent perineal lipoblastoma in a 2.5-year-old boy, which showed maturation of the lipoblasts as compared to the primary tumor.

Adenomyolipoma of the uterus: a case report.

Adenomyolipoma of the uterus is a rare, benign, polypoid lesion considered to be of hamartomatous origin or represent an unusual type of benign Müllerian mixed tumour with a heterologous element. The authors present a case of uterine adenomyolipoma and discuss its pathogenesis. A 62-year-old woman complained of lower abdominal pain and postmenopausal bleeding. Imaging techniques revealed a solid ovarian mass and a polypoid intrauterine lesion. The frozen section diagnosis of the ovarian mass was a thecoma. A total hysterectomy and bilateral salpingo-oophorectomy were performed. On gross examination a pedunculated, polypoid lesion of 7x4.5x3cm was found in the uterine cavity. Microscopically, the polypoid lesion contained both epithelial and mesenchymal elements. The epithelial elements were endometrial glands of various size, formed by proliferative endometrial cells. The mesenchymal elements were composed of endometrial stroma, smooth muscle and mature adipocytes. Both the epithelial and the mesenchymal elements showed a benign appearance, were intermingled with each other and periglandular stromal condensation was absent. The lesion had an irregular surface. Microscopic diagnosis was an adenomyolipoma. The peculiar shape and microscopic features of this lesion suggested that it was a variant of benign Müllerian mixed tumour.

Laparoscopic resection of a large periadrenal nonmalignant pheochromocytoma.

This is, to our knowledge, the first case description of an extraadrenal pheochromocytoma located in the periadrenal fat. Pheochromocytoma is a tumor that originates in the chromaffin tissue. Extraadrenal pheochromocytomas have been described commonly in locations such as the organ of Zuckerkandle (29%); the bladder (12%); the sacrum, testis, rectum, and pelvic floor (2%); the upper abdomen in association with celiac, superior mesenteric, and inferior mesenteric ganglia (43%); the thorax (12%); and the neck (2%), most commonly in association with the ninth or tenth cranial nerve ganglion. Our patient was a 40-year-old woman known to have had an adrenal mass for the last 4 years. She was referred for surgery because of an increase in the size of the mass to 11 cm. Laparoscopic adrenalectomy was performed via a posterior flank approach. The pathology report was of periadrenal fat pheochromocytoma, with positive staining for synaptophysin, chromogranin, and vimentin. The patient was discharged on postoperative day 3. The unique feature in this case was the uncommon location of the extraadrenal tumor: the supraadrenal fat. The other unique finding in this case was that the pheochromocytoma was neither symptomatic nor malignant, common features of extraadrenal masses.